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ON 

CHOREA AND CHOREIFORM AFFECTIONS 



Digitized by the Internet Archive 

in 2010 with funding from 

Open Knowledge Commons and Harvard Medical School 



http://www.archive.org/details/onchoreachoreifoOOosle 



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AND 



CHOREIFORM AFFECTIONS 



BY 

WILLIAM OSLER, M.D. 

Fellow of the royal college of physicians, London ; president of the association 
of american physicians ; professor of medicine johns hopkins university, 

and physician-in-chief johns hopkins hospital, baltimore> 

formerly physician to the infirmary for diseases of the nervous system, 

philadelphia 



PHILADELPHIA 

P. BLAKISTON, SON & CO. 

No. 1012 WALNUT STREET 
1894 




/sys 




To W. R. GOWERS, M.D., F.R.S. 

Dear Gowers, 

To the profession of the United States and Canada you stand 
as the most brilliant British exponent of the complex science of 
neurology. 

Please accept the dedication of this little volume as an earnest of 
the gratitude felt towards you by thousands of your kinsmen across 
the water, and as an expression of the personal attachment of 

Your sincere friend, 

The Author. 



" Tout est extraordinaire dans cette Maladie ; son nom est ridicule, ses 
symptomes singuliers, son caractere equivoque, sa cause inconnue, son traitement 
probl^matique." 

— BOUTEILLE, in Preface of his " Traits de la Choree," Paris, 1810. 

" Vix datur morbus ullus qui to ties medicorum de sua natura et indole 
illuserit judica ut Chorea Sti. Viti." 

— Bernt, " Monographia Chorese Sti. Viti," Prague, 18 10. 



PREFACE 



A very large part of the material utilized in these studies is 
from the case-books of the Infirmary for Diseases of the Nervous 
System, Philadelphia. The sections on the general etiology and 
symptomatology of Chorea minor represent, in expanded form, the 
Lectures on Chorea which appeared in the Medical News, 1887. 
The chapter on The Heart in Chorea is also based on the 
Infirmary cases and on a careful study of a series of autopsies 
collected from the literature since 1881. 

I have to express my obligations to my former colleagues, Dr. 
Weir Mitchell, Dr. Wharton Sinkler, and Dr. Morris J. Lewis, 
who permitted me to use their cases in preparing the statistical 
details of the 554 cases of chorea minor which are in the books 
to May 1st, 1889; also to Dr. Charles Burr and to Dr. Caspar 
Sharpies for great assistance in the tabulation of the cases, and 
more particularly for their aid in the study of the condition of 
the heart in old patients. 

Johns Hopkins University, 
July 1st, 1894. 



CONTENTS 



PAGE 

Dedication . - v 

Preface vii 



INTRODUCTION. I 



CHAPTER I. 

Historical Note — Etiology, Age, Sex, Race, Seasonal Relations, Imitation, 
Trauma, Reflex Irritation, Eye-strain — Rheumatism (Arthritis) — Infec- 
tious Diseases — Hysteria — Poisons 3 

CHAPTER II. 

Symptoms. 

Mild, Severe, and Maniacal Forms — Chorea Insaniens-^Special Symptoms 

— Motor Disturbances — Muscular Weakness ..... 20 



CHAPTER III. 

Symptoms ^continued). 

Disturbances of Sensation — Headache — Mental Symptoms — Cutaneous 
Symptoms— Urine, Nephritis — Fever — Duration — Recurrence — Chorea 
in Pregnancy — Chorea Minor in the Aged 33 

CHAPTER IV. 

The Heart in Chorea Minor. 

Condition of the Heart during the Attack — Condition of the Heart in Fatal 
Cases — Subsequent Heart History in Choreic Patients — Pericarditis in 
Chorea 47 



X CONTENTS. 

CHAPTER V. 
Morbid Anatomy and Pathology — Treatment. 

PAGE 

Statistics of Fatal Cases — Changes in the Nervous System — Bacteriology — 

Pathology — Treatment ......... 60 

CHAPTER VI. 

Choreiform Affections — Habit Spasms— Tic. 

i. Simple Tic — Habit Spasm or Habit Chorea — Generalized Tic. ii. Tic 
with Imperative Ideas, &c. iii. Complex co-ordinated Forms of Tic. 
iv. Cases of Noisy Spasm of Respiratory Muscles .... 72 

CHAPTER VII. 

Chronic Progressive Chorea (Huntington's Chorea). 

Historical Note — Report of Two Families — Special Symptoms — Morbid 

Anatomy — Diagnosis 96 



APPENDIX: Table of Recent Autopsies . . . . .113 




ON 



H O R E A 



INTRODUCTION 

In the whole range of medical terminology there is no such olla 
podrida as Chorea, which for a century _ has served as a sort of 
nosological pot into which authors have cast indiscriminately affec- 
tions characterized by irregular, purposefess movements. With 
muscular disorder as the salient feature, as the generic character, 
of chorea, there have been scores of specific designations, indicating 
the quality of the movement, the locality involved, &c. In Foster's 
Dictionary ninety-four of these sub-varieties are named. In the 
gradual growth of our knowledge of spasmodic affections this con- 
fusion has been perhaps inevitable. Even to-day it is not possible 
to make a satisfactory etiological classification, and the best we can 
do is to separate certain well-defined clinical forms, to which we 
may attempt to limit the use of the term chorea. 

The epidemic disorder of motion which Paracelsus called Chorea 
Sancti Viti has a sort of prescriptive right to the name. Sydenham's 
error in adopting it for an affection of a totally different nature has 
been condoned by two centuries of usage ; so that to these two 
forms, known respectively as chorea major and chorea minor, and 
each as St. Vitus's Dance, the name will doubtless cling. Then 
comes a long series of motor disorders in which the term has been 
freely used — the habit spasms and the various forms of tic so often 
confounded with chorea minor, the so-called symptomatic choreas, 
the chronic, the hereditary, the congenital, and the spastic forms, 
and the pre- and post-hemiplegic disorders of motion. 

B 



2 INTRODUCTION. 

These various affections may be grouped and defined as fol- 
lows : — ■ 

Chorea Minor— Sydenham's chorea— an acute disease of child- 
hood, rarely of adults and of the aged, characterized by irregular, 
involuntary movements, a variable amount of psychical disturbance, 
and associated very often with arthritis and endocarditis. The 
disease is usually regarded as a neurosis, but the clinical characters 
of the severer cases, and the frequent heart and joint implication, 
have suggested to many recent writers that it may be due to a 
specific poison. 

Chorea Major, under which term are now embraced both the 
dancing mania and the various forms of rhythmical or hysterical 
disorders of motion. Psychical impressions, emotional disturbances, 
and imitation play the most important role in this form. 

Choreiform Affections or Pseudo-Choreas. — The various 
forms of habit spasm or tic, local or generalized, which are perhaps 
best grouped under the latter term, in the more extended use as 
employed by the French. 

Secondary or Symptomatic Choreas. — Chronic disorders of 
motion, which depend upon degenerative and irritative lesions of 
the motor cortex or path. Here may be included the pre- and 
the post-hemiplegic disorders of movement, the so-called spastic 
choreas, and many of the cases of congenital and chronic chorea. 
One malady alone in this group may be separated as an indepen- 
dent affection, viz., the chronic progressive form, the so-called 
Huntington's chorea. 

I shall not consider here chorea major, which belongs to hysteria, 
or the varieties known as symptomatic, except the chronic progres- 
sive form. 



CHAPTER I. 

HISTORICAL NOTE. ETIOLOGY. 

Historical Note — Etiology, age, sex, race, seasonal relations, imitation, trauma, 
reflex irritation, eye-strain — Rheumatism (arthritis) — Infectious diseases — 
Hysteria — -Poisons. 

Historical Note. — The recognition of chorea minor as a dis- 
tinct disease dates from Sydenham, whose Schedula Monitoria 
(1686) and Processus Integri (1693) contain brief but very 
accurate accounts of the affection as we now know it. His 
description in the Schedula Monitoria is as follows : — " S't. Vitus's 
dance is a sort of convulsion which attacks boys and girls from the 
tenth year until they have done growing At first it shows itself by 
a halting, or rather an unsteady movement of one of the legs, 
which the patient drags. Then it is seen in the hand of the same 
side. The patient cannot keep it a moment in its place, whether he 
lay it upon his breast or any other part of his body. Do what he 
may it will be jerked elsewhere convulsively. If any vessel filled 
with drink be put into his hand, before it reaches his mouth he will 
exhibit a thousand gesticulations like a mountebank. He holds 
the cup out straight, as if to move it to his mouth, but has his hand 
carried elsewhere by sudden jerks. Then, perhaps, he contrives to 
bring it to his mouth. If so, he will drink the liquid off at a gulp ; 
just as if he were trying to amuse the spectators by his antics. 

"Now this affection arises from some humour falling on the 
nerves, and such irritation causes the spasm." 1 

During the fourteenth, fifteenth and sixteenth centuries, under the 
influence of religious excitement, there were epidemics of dancing 
mania in Germany and the Netherlands. Pilgrimages were made to 
various shrines in search of relief, and as that of St. Vitus, in Zabern, 
was especially famous the dance became known by his name. 
Elsewhere, from other shrines, the disease received other names, 

1 Sydenham's Works. Latham's translation. Vol. II, p. 198. Sydenham 
Society's Edition. 

B 2 



4 CHOREA. 

as St. John's, or St. Anthony's dance. The epidemics gradually 
died out, but, curiously enough, as Charcot states, an annual pil- 
grimage of dancers, in perpetuation of the custom, is still made to 
the church of St. Willibrod, possibly the same as the " procession 
of the jumping saints " which Hirsch says is still commemorated at 
Echternach in Luxemburg. It is interesting to note that in Ken- 
tucky and Tennessee, in the early part of the century, during periods 
of intense religious enthusiasm, there were epidemic convulsions, 
similar in some respects to the dancing mania of the middle ages. 
In 1805, Dr. Felix Robertson described them in an "Essay on 
Chorea Sancti Viti," in his doctorate thesis at the University of 
Pennsylvania. 

For a full and accurate description of the dancing mania, as it 
has occurred in various countries, Hecker's work 1 is still the standard 
authority. He there states that Paracelsus, in the beginning of the 
sixteenth century, made the disorder a subject of special study, and 
gave to it the name chorea. Though he made three divisions, they 
all referred to the dancing mania or to the hysterical form. It was 
unfortunate, to say the least, that Sydenham should have given the 
name chorea to an affection which had nothing whatever to do with 
the Chorea Sancti Viti, but custom has now sanctioned the use ; and 
this is not the only instance in medicine in which we know a disease 
by a name the original significance of which has long been lost. 

The modern study of the disease dates from the monographs of 
Bouteille 2 in France, and of Bernt :j in Bohemia. The work of 
Bouteille is in many respects remarkable. In addition to a very full 
and accurate historical study, he gives an admirable clinical descrip- 
tion of the disease, and makes a useful classification into essential or 
proto-pathic, secondary or deutero-pathic, and false or pseudo-pathic. 
The cases are given in great detail, and he- appears to have been 
fully aware of the association in many instances with rheumatism. 4 

1 The Epidemics of the Middle Ages. J. F. C. Hecker, M.D., Sydenham 
Society, London, 1844. 

2 Traiti de la Choree, par E. M. Bouteille. Paris, 1810. 

3 Monographic/. Chorece Sti. Viti, auctore Josepho Bernt, Med.D., &c. Prague, 

MDCCCX. 

4 The work is in another respect remarkable as the production of an octogenarian. 
In the dedication of the work to Vicq-D'Azyr he speaks of himself as octogenarius 
medicus. He was born in 1732, graduated from Montpellier, and was Government 
Physician for the study of miliary fever, on which he wrote a memoir. He prac- 
tised at Mansosque in the department of the Basses-Alpes. He wrote on many 
subjects, particularly on hydrophobia. He died in 1815, in his eighty-fourth year. 



HISTORICAL NOTE. 5 

Bernt's monograph is of special value, as it contains the older 
literature, and the reports of many interesting cases. All sorts of 
convulsive disorders are described under the name chorea. Many 
of the names still current, of varieties and sub-divisions of the dis- 
order, of which he gives thirty-one ! date from this work. 1 

In France the brilliant lectures of Trousseau, but especially the 
monograph of See in 1850, and the extensive article of Roger, were 
among the most important contributions. Charcot and his pupils 
have contributed enormously to the proper appreciation of the 
varieties of chorea, and to our knowledge of the choreiform affec- 
tions. 

In Germany Romberg, Steiner, von Ziemssen, and many others, 
have published important studies. 

In another than the usual sense, the disease may be called Chorea 
Anglorum, since by far the most important contributions have been 
made by english physicians. From Guy's Hospital have come 
many notable contributions. Bright, in Volume II. of the Reports 
on Medical Cases, 1831, gives an admirable clinical description of 
the disease, and gives a special section on chorea and rheumatism. 
He also called attention to the association of pericarditis with 
chorea. In the Guy's Hospital Reports are papers by Babbington, 
Vol. VI., 1841 ; by H. M. Hughes, 1846; by H. M. Hughes 
and E. Burton Brown, 1855 ; by Lever (on Chorea of Pregnancy), 
1847 and 1848; by Pye-Smith, 1874; and by Goodall, 1890; and 
other contributions have been made by Wilks and Hilton Fagge. 
Of other British writers who have contributed extensively to our 
clinical and anatomical knowledge of the disease, may be mentioned 
Todd, Begbie, Kirkes, Ogle, Hughlings-Jackson, Broadbent, Dickin- 
son, Tuckwell, Bastian, Sturges, Duckworth, Gee, Gowers, Money, 
Stephen Mackenzie, and Herringham. In the United States there 
have been reports of cases and careful studies by Levick, H. C. 
Wood, Mills, Jacobi, Hamilton, Haven, Putnam, and others ; while 
from the Philadelphia Infirmary for Diseases of the Nervous System, 
a large number of important contributions have been made by Weir 
Mitchell, Sinkler, Gerhard, Allison, Morris J. Lewis, and De 
Schweinitz. 

1 Bernt was born in 1770, graduated from Prague in 1797, was made Professor of 
Forensic Medicine in Prague in 1808. In 1813 he was called to Vienna to the 
same chair. He was the author of many works on Medical Jurisprudence. 



6 CHOREA. 

GENERAL ETIOLOGY. 

The records of the Philadelphia Infirmary for Diseases of the 
Nervous System to May ist, 1889, contain 554 cases, upon which 
the statements here made are based, supplemented to some extent by 
reference to cases at the Dispensary for Diseases of the Nervous 
System, Johns Hopkins Hospital. 

Sex. — Females are attacked in the proportion of rather more than 
two to one; thus of 554 cases 161 were males, and 390, about 
70 per cent., were females. In three the sex was not noted. This 
is a lower proportion than is given by many authors. After puberty 
the preponderance of females is much greater than in children. 

Age. — It is essentially a disease of childhood and adolescence. 
The age incidence of 522 cases is given in the following table : — 





H 


Un- 
der 


































Over 






H 


4 


5 


6 


7 


8 


9 


10 


11 


12 


13 


14 


15 


16-20 


21-25 


26-30 


31-35 


36-40 


40 


Males . 


J 37 


7 


3 


10 


11 


9 


20 


11 


21 


2 


J 4 


5 


11 


11 


1 








1 


Females . 


38S 


11 


12 


IS 


28 


42 


43 


39 


37 


29 


19 


23 


25 


5i 


5 


4 


1 




1 




522 


18 


15 


25 


39 


Si 


63 


5° 


58 


3i 


33 


37 


36 


62 


6 


4 


1 




2 



Arranged in decades the figures are as follows : — 




First decade ...... 


261 


Second decade ...... 


248 


Third decade ..... 


10 


Fourth decade ..... 


1 


Above fourth decade .... 


2 


Arranged in hemi-decades the figures are : — 




First hemi-decade .... 


33 


Second hemi-decade .... 


228 


Third hemi-decade .... 


212 


Fourth hemi-decade .... 


62 



It is thus seen that the second and third hemi-decades are the 
periods in which occur more than three-fourths of the entire number 
of cases. In females the age incidence is somewhat earlier than in 
males. The second hemi-decade contains the greatest number of 
•cases in males, and the third the greatest number in females. The 



GENERAL ETIOLOGY. J 

age incidence seems to be somewhat earlier in the United States 
than in Great Britain. In the report of the Collective Investigation 
Committee of the British Medical Association, 1 by Stephen Mac- 
kenzie, of the 439 cases analyzed, the largest proportion occurred in 
the third hemi-decade. Practically the disease is rare under the 
fourth year. The Infirmary records include two cases which are 
stated to be congenital, but I believe that such are in all instances 
associated with definite cerebral changes, and belong to an entirely 
different disease. It would be extremely difficult to distinguish 
between the jerky, irregular movements of an infant, and those seen 
in infants with meningeal haemorrhage, or the subsequent changes 
induced thereby. 

The ratio of cases at the Infirmary to other diseases was i to 
1 80. 

Station in Life. — The disease affects children of all grades of 
society. It is, however, more common among the artisan and lower 
classes. Our clinical information about the disease has been drawn 
almost exclusively from hospital work. In the Collective Investiga- 
tion Report, already referred to, the returns which were furnished 
chiefly by general practitioners show 72*27 per cent, of cases 
belonging to the lower classes. 

Race. — Chorea is rare in the negro, as shown by the inquiries 
instituted by Weir Mitchell some years ago among physicians in the 
Southern States. Sinkler, who has also written on this question, 
has seen but one case in the full-blooded negro. The records 
of the Infirmary show that no negro child of full blood has 
been under treatment, and only four or five cases in mulatto 
children are noted. As the proportion of coloured to white is in 
Philadelphia as 1 to 25, it would have been reasonable to expect 
a larger proportion of coloured patients, if chorea was as common 
in them as in the whites. Of 175 cases at the Johns Hopkins 
Hospital there were five in the negro race. 

The disease is also rare among the Indians. I have made 
inquiries at the Indian schools, and of a number of physicians who 
have practised for years in the American and Canadian North-west 
territories. There were several references made to its occurrence 
in half-breeds, but not one of my correspondents mentioned a case 
in the full-blooded Indian. Dr. Waldron, of the Hampton Normal 
and Agricultural Institute, Hampton, Va., writes that he has never 
seen a case in an Indian, and that there had not been any in the 
1 British Medical Journal, 1S87, I. 



Q CHOREA. 

school since its foundation. Dr. McLelland, of the Lincoln 
Institution of Philadelphia, writes to the same effect. Dr. O. G. 
Given, Physician to the Indian School at Carlisle, Pa., states that 
during the five years in which he has had medical charge of the 
children there has only been one case, and that was in a scrofulous 
and epileptic subject. Professor Robert Bell, of the Canadian 
Geological Survey, who has studied the habits and diseases of the 
Indians from Gaspe to the Rocky Mountains, and from Lake Huron 
to Hudson Bay, has neither seen nor heard of a case. Chief Peter 
Jones, a physician who has practised for twenty years among the 
Misissaquis and Six Nation Indians at the Reserve near Brantford, 
Ontario, informed Prof. Bell that he had seen three cases in half- 
breed girls, and had heard of two others. He does not think that 
it ever occurs among the full-blooded Indians. Dr. R. M. Stephen, 
Government Physician to the Indians of Manitoulin Island, has not 
met with a case in five years' practice. In twenty-five years' experience 
in the Canadian North-West, Dr. Schultz has seen only one case, 
and that was in a half-breed. 

Seasonal Relations. — Careful studies have been made at the 
Infirmary for Diseases of the Nervous System upon the relative fre- 
quency of chorea at different seasons of the year, the results of which 
have been published by Gerhard, and by Mitchell in his well-known 
"Lectures on Diseases of the Nervous System,"and in several important 
communications by Morris J. Lewis, whose latest paper, entitled 
" A Study of the Seasonal Relations of Chorea and Rheumatism for 
a Period of Fifteen Years," analyzes as regards the months of onset 
717 separate attacks of chorea. 1 November shows the fewest 
attacks, namely, 24, or 3*3 per cent. A somewhat rapid rise takes 
place in December to 56, or 7*8 per cent. The number of cases 
remains almost stationary during January and February ; then sud- 
denly increases, reaching the highest point in March, namely, 101, or 
14 per cent. In April a fall occurs to 63, or 87 per cent. Then a rise 
takes place in May to 80, or ii'i per cent, the tracing after this 
falling gradually to its lowest point in November. Lewis' studies 
include a careful comparison of the number of attacks of chorea 
with the mean relative humidity, the mean barometer, the mean 
daily range of thermometer, and the amount of sunshine or cloudy 
weather. " The highest spring point of the chorea tracing cor- 
responds with cool weather and a low barometer and mean relative 
humidity tracing, but the rise in the autumn corresponds with cool 

1 Transactions of the Association of American Physicians, Vol. VII, 1892. 



GENERAL ETIOLOGY. 9 

weather and comparatively high barometer and mean relative 
humidity, the temperature record therefore giving us but little 
information." The conclusions drawn by Lewis from his careful 
studies, which have extended over a period of many years, and 
practically embrace all our knowledge on the subject, may here be 
given : — 

ist. — " The seasonal relationship of chorea and rheumatism is 
proven." 

2nd. — "There is a marked resemblance in form between the 
chorea and rheumatism tracings and the tracing representing the 
total amount of sickness present in the community per month." 

3rd. — " This monthly variation in amount of sickness is not a 
cause in the fluctuation in the chorea and rheumatism tracing, but 
is itself probably due to the same influence." 

4th. — "While over-study assuredly plays a most important role in 
predisposing children to chorea, the months of greatest study, and, 
therefore, presumably of the greatest depression of bodily vigour, 
do not coincide with, or even precede with any regularity, the 
months of greatest frequency of the disease." 

5th. — " It is more than probable that ' weather ' is one of the 
most important predisposing causes .... although precisely which 
meteorological factor is the baneful one does not clearly appear. 
No one element of ' weather ' explains fully the fluctuations of 
these tracings for chorea, although in the barometer and storm 
statistics the relationship appears to be closer than to any other 
etiological factor or factors that have, as yet, been advanced. It is 
as if a conclusion was attempted to be drawn from premises, some 
of which are imperfectly stated or not clearly understood, or 
possibly even overlooked." 

6th. — " Either this apparently close relationship must be acknow- 
ledged to have an important place in the etiology of these diseases, 
or else the resemblance must be considered to be purely accidental, 
which seems most unlikely from a study of the tables shown." 

Locality. — The disease prevails more in towns than in the 
country ; but it is extremely widespread, as shown by the report of 
Isambard Owen x on the distribution of the disease in Great Britain. 
My friend Dr. W. N. Gordon tells me that in Exeter the number of 
cases admitted to the wards is proportionately very large. 

Family Disposition. — -It is not uncommon to find a special 

1 Transactions of the International Medical Confess, Washington, "Vol. V, 
p. 157. 



IO CHOREA. 

tendency to the disease in certain families ; thus, there were eighty 
cases, in which there was a history of attacks of chorea in other 
members of the family. Now and again we find that the mother of 
the child has also had chorea, and in one instance both mother and 
grandmother had been affected. 

Temperament— It is generally admitted that this plays an 
important predisposing role. Dull, heavy, phlegmatic children rarely 
have chorea, while high-strung, excitable, nervous children seem 
specially liable to the disease. 

Psychical influences. — These have always been held to play 
an important part. In 86 cases, 15-5 per cent , fright was given as 
the exciting cause. In the majority of these no very close connection 
existed between the fright and the onset of the chorea, as usually 
an interval of two or more days had elapsed ; but in a few cases, the 
attack came on at once. Causes other than fright are often assigned, 
such as mental worry and trouble, a sudden grief, a scolding, or, in 
some instances, the excitement of a religious meeting. The strain 
in education, particularly in young girls, during the third decade, 
the period, to use Clou ston's phrase, of "co-ordination of motion and 
emotion," 1 is an important factor. Such phrases as " over-work at 
school," "worry about lessons," "examinations" occurring so 
frequently as they do in the records, are not without significance. 
Bright-eyed, intelligent, active-minded little girls from ten to fourteen, 
ambitious to do well at school, often stimulated in their efforts by 
teachers and parents, form a large contingent of the cases of chorea 
both of hospital and in private practice. Sturges, in particular, has 
called attention to this school-made chorea, as a serious injurious 
result of our modern methods of forced education. 

Imitation, so often mentioned as an exciting cause, does not 
appear to have influenced the onset in a single case in the Infirmary 
series. In institutions or in a single ward, where numbers of cases 
have occurred in rapid succession, the disease is usually hysteria. 
Steiner, 2 who describes an outbreak of chorea minor in Prague, 
could however see nothing to support the imitation theory, and 
thought that the atmospheric influences had more to do with it. In 
1880, an epidemic occurred at the Church Home for children, in 
Philadelphia, which is given at length in Weir Mitchell's Lectures, 
but in this, as in a majority of similar outbreaks, hysteria was the 
underlying condition. An interesting outbreak occurred at Wildbad 

1 Neuroses of Developmeiit. Edinburgh, 189 1. 

2 /ahrbiick fur Kinder heilkunde, 1870. 



GENERAL ETIOLOGY. II 

a few years ago and is described at length by Wichmann. 1 Some of 
the cases seem to have been true chorea minor, but a majority of 
them were examples of the rhythmic (hysterical) disorders of 
motion. 

Traumatic influences have been assigned, such as falls and blows. 
Sinkler, in his article on chorea, in Pepper's System of Medicine, 
gives a case which followed shortly after a minor surgical operation. 
In the Infirmary records, there are a good many cases with a 
history of injury preceding the attack. 

Reflex irritation. — The older writers laid much stress on so- 
called reflex chorea, which was thought to originate from digestive 
disturbances, the presence of worms — the choree vermineuse of the 
French — or genital irritation. I do not find a single instance in the 
Infirmary records to indicate any causal relationship between these 
conditions and chorea. 

Jacobi 2 has called attention to the important part played by naso- 
pharyngeal irritation in chorea, more particularly when confined to 
the face, but this habit spasm, as it is more correctly called, is in 
reality a different affection from the true chorea minor. 

Here it is worth noting that Straton 3 laid great stress upon the 
fact that erosions of the mucous membranes of the nose and throat, 
by affording portals of entry to micro-organisms, might be the 
indirect cause of chorea in children. 

Relation of Chorea minor to eye -strain.- -It has been claimed 
by Stevens 4 and others that ocular defects lie at the basis of many 
cases of chorea, and that with the correction of these the irregular 
movements disappear. To test the truth of these statements a 
careful study was made at the Infirmary by De Schweinitz as to the 
condition of the eyes in fifty cases of chorea in children with the 
following result : — Hypermetropia was present in twenty-three, or 46 
per cent. ; hypermetropia in one eye, and hypermetropic astigmatism 
in the other in seven, or 14 per cent. ; hypermetropic astigmatism in 
twelve, or 24 per cent. ; myopia in one, or 2 per cent. ; myopic 
astigmatism in three, or 6 per cent ; mixed astigmatism in four, or 
8 per cent. De Schweinitz then adds to his report the cases 
reported by Stevens and C. S. Bull of New York, making a total of 

1 Deutsche medicinische Wochenschrift. 1890. 

2 American Journal of the Medical Sciences, 1886. 

3 British Medical Journal, 1885, Vol. II. 

4 Transactions of the New York Academy of Medicine, 1874-76, and New York 
Medical Record, August, 1876. 

5 New York Medical Journal, 1887, I. 



12 CHOREA. 

227 cases, of which 112 were ametropic, and 115 emmetropic. His 
conclusions are expressed in the following paragraphs : — " Hyper- 
metropia and hypermetropic astigmatism are vastly the preponder- 
ating condition in the eyes of choreic children, being found in about 
7 7 per cent, of the cases, exactly as hypermetropic refraction is the 
preponderating condition in childhood, being found in 76 per cent, 
of the eyes of children in the elementary schools." " The evidence, 
however, seems quite as lacking, that hypermetropic refraction is the 
basal cause of chorea, as it is that the chorea is the cause of the 
hypermetropia." Under date of June 18th, 1894, Dr. De Schweinitz 
wrote to me stating that he has now examined more than a hundred 
cases of chorea, and he adds, " that ordinary chorea and many of 
the forms of facial spasm, habit spasm, etc., are materially benefited 
by correcting refractive errors and anomalies of the ocular muscles, 
just as they are helped by a variety of other treatments, but I do 
not believe that there is any proof to show that eye-strain of itself is 
responsible for their origin, with perhaps the single exception of the 
so-called habit spasms affecting the orbicularis and immediate facial 
area. Certainly many of these will disappear promptly after the 
refractive error is corrected without any treatment whatsoever, and 
they will not disappear if you do not relieve the eye-strain. In a 
constitution predisposed to chorea, I presume eye-strain is a very 
important factor in fostering and perhaps provoking attacks, but that 
is all." 

Of the five cases of chorea submitted to Stevens by the New 
York Neurological Society, 1 not one was chorea minor, so that the 
statements of the report are scarcely germane to the question. 

Ranney's recent report 2 upon this subject is of interest in two 
connections : — In the first place it shows the necessity of the recogni- 
tion of some uniformity in deciding exactly as to the limitation of 
Sydenham's chorea. Of these twelve cases, nine patients were 
fifteen years or older, the majority of them were adults, and the 
muscular disorder had persisted for eighteen months, the shortest 
period, to thirty-one years. I do not think that any one of the 
twelve cases could be really called true Sydenham's chorea, but that 
nearly all were one or other of the varieties of habit spasm or tic, 
some possibly of hysterical chorea. The second point is the 
enormous importance of careful examination of the eyes in these 
chronic forms of disordered imis dilation, to use Roth's term. 

1 Journal of Nervous and Mental Diseases, 1889, p. 649. 

2 New York Medical Record, May 5th and 12th, 1894. 



ARTHRITIS (RHEUMATISM). 1 3 

I know that there are circles in New York in which Dr. Ranney 
bears the reproaches of a prophet, but — litera scripta manet. 

Arthritis (Rheumatism), — The association of arthritis and 
chorea did not escape the observation of physicians in the last 
century, and Bouteille 1 gives in his monograph two observations of 
Stahl, and two of Sauvages. Bright states that in the edition of the 
" Syllabus, or Outlines of Lectures on the Practice of Medicine," 
published at Guy's Hospital in 1802, rheumatism was distinctly 
recognized as one of the causes of chorea, and he says also that in 
the later editions, as in that of 1820, it was stated that "chorea 
sometimes alternates with acute rheumatism." Bouteille makes the 
rheumatic one of the minor sub-divisions of his deutero-pathic or 
secondary chorea. So far as I can see he lays no stress on it in 
connection with the essential or primary form. Copeland 2 writes : — 
" The association of this disease with rheumatism has been observed 
by me on several occasions, and in nearly all there has been a 
marked disposition of the rheumatic affection to recede from the 
joints or extremities, and attack the internal fibro-serous membranes, 
as those of the cerebro-spinal axis, and the pericardium." J. C. 
Pritchard, 3 of Bristol, reports several severe and fatal cases of chorea, 
and speaking of one of them in which pericarditis was present, he 
says : " the disease seemed to have arisen from the metastases of 
rheumatism." The association between chorea and rheumatism 
was evidently very clearly ' recognized by the Guy's Hospital 
physicians during the early decades of this century, and Bright 4 has 
directed particular attention to this question ; but in Babbington's 
paper on Chorea in the Reports, 5 no special stress is laid upon 
rheumatism. He remarks that " rheumatism also, when it affects 
the heart and pericardium, may give rise to the disease through the 
irritation of the plexus and ganglia, which so entirely surround that 
organ, and the origin of its great vessels." Oddly enough, con- 
sidering that his paper was written only a couple of years after 
Bright's communication, he gives the credit of his discovery of the 
association of disease of the heart with chorea to Addison, stating 
that " should further investigation prove chorea to be more intimately 
dependent on disease of the heart or pericardium, than has been 

1 Opus cit., p. 291. 

2 London Medical Repository, Vol. XV. 

3 London Medical Repository, Vol. XXI, 1824. 

4 In Vol. II. of his Medical Reports, and in Medico- Chirurgical Society's 
Transactions, Vol. XXII, 1S39. 5 Vol. VI, 1841. 



14 CHOREA. 

hitherto supposed, the merit of the discovery will certainly be due 
to Dr. Addison." 

The strongest support to the rheumatic theory of the origin of 
chorea was offered by the French writers, particularly by Germain 
See 1 in 1850, and by Roger 2 in 1866. See's conclusions may be 
briefly expressed as follows : — Of two rheumatic infants at least one 
will be choreic ; and of five choreic children there are two rheu- 
matic; while "Roger concluded that articular rheumatism, chorea, 
and heart disease (endocarditis) were three terms of one and the 
same pathological state or phase — la choree rheitmato-cardiaque. 
Since the appearance of these papers every writer on the subject has 
dealt more or less fully with the question of the relation of the two 
disorders/' The statistical method which has been applied to the 
solution of the problem has served to bring out very striking dis- 
crepancies. The German writers, as a rule, have not placed very 
much stress upon rheumatic relations of the disease. Thus Steiner, 
of Prague, found only four instances of acute arthritis in 252 cases 
of chorea. English authors place the coincidence very much higher, 
and make it from 20 to 50, and even 70 per cent. 

So far as the statistics are of any value, the following statements 
may be made with reference to the relations of arthritis and chorea 
in the United States. Of the 554 cases at the Infirmary for Diseases 
of the Nervous System, Philadelphia, in 15-5 per cent, there was a 
history of rheumatism in the family; eighty-eight cases, 15*8 per 
cent , had had at some time or other, either prior to, with, or sub- 
sequent to the attack of chorea,- acute or subacute articular swellings. 
In thirty-three cases there was a definite history of pains in various 
parts, sometimes described as rheumatic, but not associated with 
joint trouble, and if these are regarded as rheumatic, and added to 
those with manifest arthritis, the percentage is raised to nearly 21. 
At the Johns Hopkins Hospital, of 175 cases treated in the wards 
and in the Dispensary to January 1st, 1893, there was a history of 
arthritis, acute or subacute, in twenty-seven cases, or in 18*24 P er 
cent. 

At the discussion which took place before the American Pediatric 
Society at Boston, 4 Charles W. Townsend reported 148 cases with a 

1 Mhnoires de P Academie de Medicine, Tome XV, 1850. 

2 Arch. Gin. de Midicine, December, 1866, and January, 1867. 

3 In any discussion on this point, arthritis and rheumatism must be regarded as 
convertible terms, though the joint affections of chorea may be no more rheti- 
matic than those of cerebro-spinal meningitis. 

4 Transactions of the American Pediatric Society, Vol. IV, 1892. 



ARTHRITIS (RHEUMATISM). I 5 

history of rheumatism in thirty-one, or 21 per cent. ; Crandall, 
eighty-eight cases with rheumatism in some form, either before or 
after the chorea, in 54 per cent. ; M. Allen Starr, 385 cases, with a 
history of rheumatism in 18 per cent., growing pains or pain of an 
indefinite kind not being included under this head. In the discus- 
sion which followed, Jacobi took strong grounds in favour of the 
close relationship of the two affections, an opinion supported strongly 
also by Holt. No unanimity of opinion was reached on the ques- 
tion ; the only very high rheumatic percentage was that given by 
Crandall. Of course, in making up statistics, unless the histories 
have been taken with actual reference to the points under discussion, 
not much reliance can be placed upon them, and it is this very fact 
which makes the figures from the Infirmary for Diseases of the 
Nervous System of special value, inasmuch as the chorea histories 
have been taken in special books provided with full and detailed 
printed questions, particularly on the possible rheumatic manifesta- 
tions. Of the last 144 cases of the Infirmary series, in almost every 
one of which I myself made the most careful inquiries of the rela- 
tives as to rheumatic features, there were only twenty-five with 
articular pains or swelling, and only six of these had had acute 
inflammation of joints. In England, among the more recent 
statistical investigations may be mentioned the report of the Com- 
mittee on Collective Investigation of the British Medical Associa- 
tion, which gives, out of a total of 439 cases, ninety-seven with a 
rheumatic history, a little more than 22 per cent., with which the 
largest collected American statistics closely agree. Of the seventy- 
three fatal cases collected by me (see Appendix) twenty-nine are 
stated to have had rheumatism, three had the subacute form, in 
four it was not definitely stated, and thirty-seven had not had acute 
arthritis. 

As insisted upon, especially by See and by Roger, the arthritis in 
a very large proportion of all cases precedes the chorea, which 
develops with its subsidence, or may not follow until con- 
valescence has been well established. In other instances the chorea 
precedes the rheumatism. This, however, is rare. In the report of 
the Collective Investigation of the British Medical Association on 
rheumatism, analyzed by Whipham, chorea was found to have pre- 
ceded rheumatism in less than 2 per cent, of the cases. 

The manifestations of rheumatism in childhood are extremely 
varied, and often so slight that they are readily overlooked. I well 
remember, in 1872, at the University Hospital, a bedside talk of 



l6 CHOREA. 

Sir William Jenner's on a case of a little girl with endo-peficarditis, 
and only the most trifling articular manifestations, which had been 
entirely overlooked before her admission. The remarks made an 
indelible impression upon me, and I have been in the habit of 
seeking carefully for the minor manifestations of the disease. The 
London physicians have done much to broaden the conception of 
rheumatism in childhood, of which subject Cheadle's l lectures give 
a full presentation. 

Without having the facts upon which to base a positive statement, 
I am of the belief that rheumatic fever is not nearly so frequent in 
Philadelphia and Baltimore as in London. Here, at any rate, some 
of the most striking rheumatic manifestations are conspicuous by 
their absence ; for instance, a case of subcutaneous fibroid nodules 
is a great rarity. 2 In my five years' service at the Infirmary for 
Diseases of the Nervous System not a single instance was seen. 
Since 1881, when I saw cases at the Great Ormond Street Hospital, 
I have been in the habit of looking for them in a case of rheumatism 
as systematically as I examine into the condition of the heart. I 
have seen a larger number of instances in adults than in children. 
The matter of their rarity was a subject of comment among the 
members of the Pediatric Society at the Boston meeting in 1892. 
They may exist independently of acute arthritis, or even of any 
rheumatic manifestations. I have had in my wards for three years 
a girl with chronic valve disease — mitral and tricuspid — who has 
never had, so far as can be ascertained, any signs of rheumatism. 
During the past eighteen months she has developed many sub- 
cutaneous fibroid nodules about the elbows and hands, and along 
the tendons in various places. As I write (June 30, 1894), there 
has been admitted to the wards the first case which I have seen 
in this country with the combination of chorea, arthritis, sub- 
cutaneous fibroid nodules, and mitral endocarditis. The only case 
which, so far as I know, has been reported in the United States 
with this combination is the remarkable one by C. H. Brown. 3 

Infectious Diseases. — With reference to its development in, or 
as a sequence of, other diseases the following statement may be 
made. Scarlet fever with arthritis may be a direct antecedent. 

1 The Rheumatic State in Childhood. London, 1S89. 

2 So far as I know the first case reported in the United States was from my 
clinic at the University Hospital, Philadelphia, by Dr. J. K. Mitchell ( Univ. 
Med. Magazine, Vol. I.) 

3 fownal of JVervous and Mental Diseases, 1S93. 



INFECTIOUS DISEASES. 1 7 

Chorea developed in three of 533 cases of scarlet fever reported by 
Carslaw. 1 A previous history of this disease was obtained in 141 
of the Infirmary cases, but in not one was the sequence immediate. 
Sturges states that a history of previous whooping-cough is met with 
very frequently in choreic children. The disease may develop 
acutely in the course of gonorrhoea, secondary syphilis, and in septic 
infections, such as puerperal fever, pyaemia, and multiple suppura- 
tive polyarthritis. In Litten's important paper 2 full details are given 
of cases associated with these acute infections. Cases have also 
been reported as occurring after diphtheria, measles, scarlet fever, 
and typhoid fever. Kinnicutt and others have reported instances 
of the association of chorea with malaria, a connection probably 
accidental and not causal, since chorea does not prevail to a greater 
degree in malarial regions. With the exception of acute polyarthritis 
(rheumatic fever) and certain forms of septicaemia there is no very 
intimate relationship between chorea minor and the infective dis- 
orders. Canine chorea also follows an acute infectious disease # of 
the dog. 

On the other hand the acute exanthems developing in the course 
of chorea usually check the disease (Rilliez and Barthez). On this 
point Radcliffe's remarks are worth quoting : " At any rate, there 
are many cases on record of measles, scarlet fever, rheumatic fever, 
or some other fever, being developed during the course of chorea, 
and in which the choreic symptoms have been suspended during 
the fever. I have met with seven such cases. Indeed, as far as I 
have had the opportunity of judging, the constant rule appears to 
be, that the chorea is aggravated in the initial stage of the fever — 
that is, in the cold stage, or stage of irritation — and suspended 
more or less completely when the stage of reaction, or hot stage, is 
established ; and that, in relation to rheumatic fever, the place of 
chorea is either before the fever (often a long time before) or after 
the fever (often a long time after) ;" s and he quotes the maxim of 
Hippocrates, "febris accedens solvit spasmos." 

S. West reports the case 4 of a child, aged ten, with chorea, in 
whom the onset of typhoid fever checked the movements. Trousseau 
also reports illustrative cases. 

Other Diseases. — Anaemia, upon which as a causal factor much 
stress has been laid, appears to be more often a sequence than an 

1 Glasgow Medical Journal, 1891. 2 Chariti Annalen, Bd. XL 

3 Reynolds' System of Medicine. Article Chorea. 
* St. Bartholomew 's Hospital Reports, Vol. XXII. 

C 



1 8 CHOREA. 

antecedent, and though cases do develop in children who are anaemic 
and in poor health, this is by no means the rule, and my experience 
lends no support to the view warmly advocated, among others by 
Rachford, that an impoverished blood condition plays an important 
role in the causation of the disease. I have, however, seen several 
instances in which chorea developed in chloritic girls at puberty. 

Litten 1 quotes a case of Roeser in which chorea developed six 
days after a copious bleeding at the nose, and proved fatal. Fresh 
endocarditis was found at autopsy. He speaks also, but gives no 
details, of two cases of chorea developing in the course of pernicious 
anaemia, both of which ended fatally, and presented at the autopsy 
signs of a fresh endocarditis. 

The Relations of Chorea and Hysteria. — In the first place, 
it is not uncommon to see hysterical manifestations in young girls with 
chorea, which are simply complications, and mean nothing more 
than that the disease has attacked an hysterical subject, so that we 
have a combination of the two affections. Many such instances 
are to be found in our records, but as a rule the hysterical 
symptoms were quite subsidiary. 

Secondly, it is quite possible for hysteria to actually simulate 
chorea minor. Such instances are very exceptional. A case was 
reported by Debove to the Societe Medicale des Hopitaux in 1890. 
The man, aged 21, had all the features of chorea minor ; the move- 
ments were wide-spread and excessive. Well-marked hysterogenic 
zones were determined. It was decided to try compression over the 
parts of the abdomen corresponding to the ovarian regions in 
women, and as a matter of fact the strongest possible pressure at 
these points caused the immediate cessation of the movements. 
The patient had also anaesthesia of the pharynx. The movements 
began the day after he had had a serious trouble with his fiancee 
and an attempt to commit suicide, in which, however, the rope 
broke. 

Thirdly, there is a possibility that chorea minor and the rhyth- 
mical or true hysterical chorea may coexist in the same person. Such 
an instance was reported by Seglas to the Societe Medicale des 
Hopitaux in 1891. The patient was a girl aged 15, who had had 
ordinary choreiform movements, at first of the face and of the left 
side, making continual gesticulations and incessant grimaces, and 
becoming worse, so that she was unable to speak and had all the 
characteristic features of chorea minor. Within two months, after 

1 Op. cit. 



HYSTERIA. 19 

some alternations of improvement and recurrence, the choreic 
movements improved and gradually disappeared from the face 
and from the limbs. In the left arm they continued, but no 
longer presented the characteristic inco-ordination which had for- 
merly been present. These gradually increased until there were 
marked rhythmical movements of the arm of the so-called 
"hammer" type — choree malleatoire. She had also at intervals 
attacks of laughing and crying. There was hyperaesthesia in the 
ovarian regions, and she became excessively emotional. 

A series of thirty-four cases of chorea with hysteria have been 
collected by Duchateau (Paris Thesis, 1893). 

Poisons. — There are a few observations pointing to the occur- 
rence of chorea as a sequence of poisoning by gases or medicines ; 
thus Observation IV. in Leudet's 1 paper, a man aged 61, an 
alcoholic, who had frequently made attempts at suicide, was found 
asphyxiated by the vapour of charcoal. He had on admission 
movements of flexion and extension of the right arm, quite forcible 
and marked. After recovery of consciousness, on the two successive 
days the movements persisted. There was no paralysis. They 
ceased on the 12th. This case, which is often quoted, has of 
course nothing to do with chorea ; the movements were such as is 
now well known not infrequently occur in poisoning by gases. 

Demme's case 2 is more probable : a boy, aged six, with caries of 
the cervical vertebrae, had an iodoform bougie placed in the fistula 
on August 1 st. He did not feel very well after it for a week or so. 
On the 1 8th a second was inserted, and on the following morning 
choreic movements began in the arms and legs. He gradually 
improved, and by September 15th was well. About the end of 
October the fistula was injected with iodoform again, and four times 
subsequently. The choreic movements returned. 

1 Archives Gdnirales de Medicine, May, 1865. 

2 Abstract in Schmidt's Jahrbikher> Bd. CCXXIV. 



C 2 



20 CHOREA. 



CHAPTER II. 

SYMPTOMS. 

Symptoms — Mild, severe, and maniacal forms — Chorea Insaniens — Special 
Symptoms — Motor disturbances — Muscular weakness. 

Although the disease may begin quite abruptly, as a rule the 
cases present premonitory symptoms, motor or psychical. The 
child is restless and unable to sit still, a condition well expressed by 
the term "fidgets," often used by the mothers in describing the 
onset. The entire disposition may be changed, and the child 
becomes irritable, cross and unmanageable. Emotional dis- 
turbances are common, the child crying on the slightest provoca- 
tion. Night terrors have been noted in many instances. Punish- 
ment inflicted at this stage by thoughtless parents or teachers 
aggravates the mischief, or even precipitates the attack. The 
appetite may fail, and anaemia not infrequently develops. Headache 
is a not uncommon complaint. Less frequently there may be at this 
stage pains in the limbs, usually in the legs, or about the joints. 
After a week or more of these symptoms the restlessness increases, 
and the sudden involuntary movements characteristic of the disease 
begin. These are often first noticed at table, when the child spills 
a tumbler of water by a sudden jerk, or upsets a plate, or drops 
something. Beginning as a rule in the hands and arms, the 
brusque, involuntary movements give a very characteristic stamp to 
the muscular disorder. In any large series three groups of cases 
may be recognized ; the mild, in which the affection of the muscles 
is slight, the speech not seriously involved, and the general health 
not much disturbed ; the severe, in which the movements are 
general, the power of speech is lost, and the patient is unable to 
get about and help himself; and the maniacal form, chorea in- 
saniens, characterized by profound cerebral disturbances. 

Mild Chorea. — A very large proportion of all the cases belong 
to this group. The symptoms are annoying, and perhaps distress- 
ing, but never alarming, and the child is always able to walk about 



MILD CHOREA. 21 

and to talk. Sometimes, however, with very slight motor symptoms 
the psychical features may be marked, showing themselves not often 
in mania, but in mental depression or even melancholia. The fol- 
lowing case is a good illustration : — 

Acute Rheumatism, followed by Chorea, Second Attack the folloiving 
Year ; Admission in Third Attack, no Heart Complication ; 
Rapid Recovery. 

Ida A., aged seven, was admitted to hospital April 24th, 1887, 
in her third attack of chorea. The mother has rheumatism. Early 
in January, 1885, the child had an attack of acute rheumatism ; was 
in bed four weeks. One month after convalescence the chorea 
began, and for several months she twitched, chiefly on the right side. 
On January 29th, 1886, she had a second attack, and attended at 
the clinic for three months. At this time there was no rheumatism, 
and the heart-sounds were clear. About the end of March of this 
year, her mother noticed that she was extremely nervous and 
irritable, and on the 30th she had irregular jerky movements of 
both hands and of the shoulders, with an occasional facial grimace. 
On April 20th she returned very much worse ; the movements of 
the arms constant, speech not affected. She was admitted to 
hospital, put to bed, given Fowler's solution, m. v, t. i. d., galvanism 
to the spine, and massage of the limbs daily, and ordered to be 
kept quiet and undisturbed. There was no heart murmur. She 
improved rapidly, and at the end of a week the movements had 
almost ceased. On May 7th she was discharged well. 

The case is a very characteristic one. A child of a family with a 
rheumatic history, had an attack of rheumatic fever, during con- 
valescence from which choreic movements began, and continued 
for several months ; then came the spring recurrence in two succes- 
sive years, which is so marked a feature in many cases. 

Severe Chorea.— Cases belonging to this category are very much 
less common, but any mild attack in which the child is able to get 
about and to talk plainly, may develop into the severer form, in 
which the muscles are so involved' that volitional movements are 
impossible, and the power of speech is lost. No more distressing 
disorder of childhood is to be seen than this severe type of chorea. 
Fortunately the cases are not very common, and in children the 
disease does not often pass into the most intense grade, in which 
there is active mania and high fever. 

The following is a good illustrative case : — 



22 CHOREA. 

History of Fright ; General Chorea, Movements Violent and Inces- 
sant, no Heart Complication. 

Ella A. (sister of the preceding), aged nine. Has not had 
rheumatism. Has been nervous at school. Failed in some lessons. 
Was frightened at a lecture at the Wagner Institute, and three 
months ago was much upset by seeing a child injured and bleeding. 
The twitching was first noticed in right arm. When brought to 
clinic, December 30th, she presented the appearance of a rosy, 
plump child. The condition was noted as follows : — Choreic move- 
ments are universal ; the hands and feet jerk about in a most 
violent manner, the movements usually beginning in the left side, 
and extending to the right. They number from thirty to thirty-two 
in a minute, and are so strong that the most powerful restraint fails 
to repress them entirely. The hands are drawn from the lap 
violently upward, striking the chest, or they rise suddenly and fall 
heavily on the knees. When she is seated on a chair the feet are 
lifted from the floor and come down suddenly with a loud stamp. 
The face is in constant movement ; speech is almost impossible, and 
she presents a truly pitiable condition. So violent are the move- 
ments that she has frequently injured and bruised herself, and she 
has several times inadvertently struck her mother. She is intelligent, 
sleeps well, even soundly, the movements ceasing completely. It 
was impossible to examine the heart on account of the extreme 
jactitation. She took Fowler's solution, up to vr^ xv, t. i. d., with 
benefit ; but the case was protracted, and although the violent 
movements subsided, it was several months before they disappeared. 

Such cases are often carried into the clinic, in some instances 
covered with bruises on the exposed and prominent portions of the 
body. 

Chorea Insaniens. — No stronger point in favour of the 
existence of a distinct and essential malady, chorea, is to be found 
than the occurrence of cases of great severity, which represent, so 
to speak, the malignant manifestations of the disease, comparable to 
those met with in other acute affections. Just as we have instances 
of very mild scarlet fever with trifling symptoms, and all grades of 
the disorder up to the scarlatina maligna, so in any large series of 
cases of chorea, the grades of the affection pass insensibly from the 
milder, trifling forms to types of such intensity that they equal in 
severity the more aggravated forms of acute nervous disorders, such 
as tetanus, hydrophobia, and cerebro-spinal fever. Maniacal chorea 



CHOREA INSANIENS. 23 

— the chorea insaniens of Bernt — is a truly terrible affection. The 
following are the only two cases which I have seen : — 

History of Fright, Rapid Onset of Chorea, Delirium, Fever, Death on 
the Tenth Day, Acute Endocarditis. 

Emma M., a strong girl, aged eighteen, was admitted to the 
Montreal General Hospital, October 17th. 1 She was a waitress at an 
hotel, and when carrying a tray was badly frightened by two men 
who were fighting, and dropped her dishes. A severe reprimand 
only increased her fright. The next day she packed her trunk and 
went home, a distance of thirty miles. On the way she met her 
father, who insisted that she should return to Montreal. By this 
time her hands and arms were in constant motion. On the way 
back her father got drunk, and threatened to punish her if she 
did not keep quiet. She was admitted five days after the first fright. 
The arms and legs were in constant motion, jerking about in all 
directions ; the face was also affected. She was rational, but could 
scarcely speak. On the night of the 19th she did not sleep, but 
raved and talked all the time ; the movements were incessant. On 
the 20th, 21st and 22nd, this condition persisted, or rather grew 
worse; the temperature ranged from 101° to 103°; the tongue was 
dry and cracked, and she became much exhausted. On the night 
of the 22nd the temperature rose to 105 , and she died five days 
after admission, and ten days from the onset of the symptoms. The 
autopsy showed recent soft, warty vegetations on the auricular 
surface of the mitral segments. (See Morbid Anatomy.) 

Rheumatism at 16 and 23, the last Attack followed by Chorea. Onset 
without known cause of General Chorea, Admission at end of 
First Week. Fever, Skin Rash, probably Secondary Syphilide. 
Delirium, Parotitis, Cyanosis, Exhaustion and Death. 
Endocarditis, Broncho-pneumonia. 

Miss A., aged 27, a teacher by occupation, was admitted to the 
Johns Hopkins Hospital on December 24th, 1889, with irregular 
movements of the extremities and body. 

Family History. — The patient has three brothers and three 
sisters living, and in good health. A brother died of pneumonia 
when young ; he had chorea for a month at the age of ten years. 

1 Case reported by Dr. Geo. Ross, Canada Medical and Surgical Journal, 
Vol. XL 



24 CHOREA. 

Her grand-parents lived to an advanced age. There is no further 
family history of import. 

Previous History. — She has been generally strong and healthy ; 
had scarlet fever, diphtheria and measles, when a child. Men- 
struated at the age of sixteen. About eleven years ago she had an 
attack of rheumatism, lasting four weeks ; the shoulders and hands 
were then swollen. She recovered her health completely afterwards. 
Since that time she has been teaching, and studying excessively 
hard, so much so that at times her friends feared she would lose her 
reason under the mental strain. A second attack of rheumatism 
occurred four years ago, after a period of intense application to her 
studies. The hands were much swollen, the other joints slightly so ; 
all were exquisitely tender. She had been ill some days, when, 
owing to some excitement, her mind became affected, and for five 
weeks she was delirious. At this time she was supposed to be 
suffering from meningitis. During three of these weeks she had the 
same irregular movements of the legs and arms as in the present 
illness, though they were not so violent. These muscular jerkings 
and mental symptoms disappeared, and she continued in good 
health until August, 1889, when the headaches, which had been 
troubling her slightly during the previous winter, became more 
severe. The eyes now often pained her, so that she could not read 
for any length of time. The back also felt weak after very slight 
exertion, and she became very nervous. Shortly afterwards she was 
confined to bed for three weeks, at the end of which time she went 
away on a visit. During this visit she was able to go about, ride 
and dance, though still suffering from headache at times. During 
the night of December 17th, 1889, she was awakened by a jerking 
of the muscles of the right arm ; then those of the other arm and 
finally the legs, face, and body became involved. This condition 
has continued unchanged until admission. 

Present State. — She is unable to sleep. The mind is clear. 
Articulation is difficult. She tosses continually from one side of the 
bed to the other ; the movements are very violent. Nutrition is 
good, as is also the colour of the skin. There are well-marked and 
very varied choreic movements, affecting all parts of the body. On 
the face and arms, here and there on the legs, and sparingly on the 
trunk, there is a flat, papular, bronze-coloured rash. In places it is 
distinctly scaly. It is most abundant on the inner side of the wrists, 
on the palms of hands and bends of elbows. It is symmetrical. 
The cervical glands are enlarged, hard, and nodular • there is slight 



CHOREA INSANIENS. 25 

enlargement of the inguinal glands on the left side. The pharynx 
is ulcerated ; uvula healthy. Careful examination of the fauces was 
not possible on account of the movements of the head. There is 
some patchy alopecia. The heart-sounds are clear, both at apex 
and base ; the apex beat is in the fifth intercostal space. On the 
sides and under surface of the clitoris is an elevated and excoriated, 
non-indurated sore. A further examination of the vagina and 
cervix was not possible. There is no incontinence of urine or of 
faeces. 

December zZth. — The patient was very restless up to midnight, and 
then, frightened by an imaginary something, she jumped out of bed, 
shrieked wildly, and apparently did not recognize the nurse. She 
was afterwards quiet for a few minutes at a time, but had periods of 
maniacal excitement, starting at every sound. The muscular move- 
ments are not so exaggerated as during the previous night, but the 
mental condition is decidedly worse. At 3.50 a.m. chloralamide, 
grs. xxx, was administered, and she became more quiet, and at 
4.30 a.m. was sleeping heavily. The choreic movements ceased 
during deep sleep. 

December 2 9 th.— Patient is somewhat better this morning. The 
muscular contractions are not so violent. The heart-sounds are 
clear, both at apex and base. There is no joint tenderness. Several 
hypodermic injections of morphia were given during the evening ; 
under its influence she slept from 9 p.m. until 1 a.m. Was then 
restless until after 3 a.m., when she slept for three-quarters of an 
hour. 

December 30th. — The choreic movements are quite general this 
morning ; the. left arm, which was quiet yesterday, is now tossed 
about equally with the right. There is less mental confusion — 
patient answering questions more rationally. The tongue is moist 
and furred. Takes nourishment better than yesterday. Under the 
influence of a third of a grain of morphia, she slept uninterruptedly 
through the night. 

December 3 1st. — Is very restless this morning. The choreic move- 
ments are about the same as yesterday. Pupils normal. She speaks 
very volubly and irregularly ; speech is not so distinct as previously. 
The tongue is moderately dry. The pulse is 144, not especially 
weak. She still takes nourishment very well by the mouth. There 
is a very marked friction erythema on the arms, face, feet, and 
knees. 

January 1st. — Patient slept more than six hours last night under 



26 CHOREA. 

the influence of morphia, given at 9.30 p.m. Pulse 100, very 
irregular. Seemed better this morning ; appetite good. Muscular 
twitchings not so violent. At mid-day she fell out of bed, and when 
picked up was found to have passed a copious liquid stool. After 
removal to bed she became somewhat cyanosed. Pulse 132, 
extremely feeble. Ether was given hypodermically, and brandy and 
hot water by mouth and rectum. At 5.30 p.m. the pulse was still 
rapid, but of better volume. She is still restless, and has occasional 
spells of shrieking. 

January 3rd. — Since the last note was taken patient has been in a 
condition of stupor, lying on back and breathing regularly, twenty- 
four to the minute. Pulse 120, not decidedly better than two days 
ago. There are occasional choreic movements, limited to the arms. 
She had several watery stools this forenoon. The abdomen is soft. 
The tongue and lips are dry and brown. At the angle of the jaw on 
the right side there is a hard and tender swelling over which the 
skin is reddened. The heart-sounds at apex and base are clear. 
There are no choreic movements. 

January /^.th. — The abdomen is soft and a little distended ; there 
are no spots visible on it. Respiration rapid, fourty-four to the 
minute. Fine crepitant rales are present in the left axillary region, 
with an expiratory rub. There is no heart murmur. The same fine 
crepitant rales are present in right axillary region, also with an 
expiratory rub. The patient sank gradually during the day, death 
occurring at 6.30 p.m. 

Autopsy, by Dr. Councilman, fourteen hours after death. 

Body of medium size, 157 centimetres long, slightly built, well 
nourished. Posterior surface much congested. Right side of face 
swollen, reddened and cedematous ; a considerable amount of fluid 
blood flowed from the ear. Bloody fluid also escaped from the 
nostrils. On the anterior surface of the right thigh, and on the 
internal surface of the left near the knee, are depressed cicatrices. 
Here and there over the body, generally corresponding to creases in 
the clothing, are congested areas. 

Cranium. — Scalp thin and firmly adherent. Skull of ordinary 
thickness, symmetrical. External surface of dura mater smooth. 
Fluid blood in longitudinal sinus. Dura slightly attached to bone 
everywhere. The brain with the meninges weighed 1,274 grammes. 
The lateral sinuses are free from clots. The petrous bone of the 
right side is hypersemic. Pia mater is slightly cedematous. The 
arteries at the bases are normal. No abnormal change is to be seen 



CHOREA INSANIENS. 2J 

in the cerebral tissues. There is no marked hyperemia of the 
cortex ; no haemorrhages. 

Abdominal Cavity.— The subcutaneous fat is slightly developed. 
Muscles red. Peritoneum smooth. The cavity dry. The liver 
and spleen are free from adhesions. The liver, spleen and pancreas 
congested. The kidneys showed fcetal lobulation. 

In Douglas's cul-de-sac are numerous ecchymoses. The uterus is 
large, blood-vessels prominent, the tissue hyperemia Small corpus 
luteum in left ovary. Immediately around the meatus urinarius, the 
mucous membrane is slightly swollen. There are no ulcerations or 
cicatrices about the genitalia. 

Thoracic Cavity. — Precordial space uncovered by lung tissue 
small. Both layers of the pericardium are smooth. The heart is 
contracted. In the right cavity a small amount of fluid blood ; 
same on left side. Heart flesh pale and flabby. On the auricular 
surface of the mitral valve are a few fresh warty vegetations. 
Columne carnee in left ventricle fibrous. Coronary arteries normal. 
The lungs are free from adhesions. In the upper part of the left 
lung is an area of consolidation, the pleura over it being covered 
with a slight fibrous exudation. On section two foci are seen, the 
centre of one of them being somewhat whitish. Adjoining them 
is a depression on the surface of the lung, and beneath this is a 
caseous focus 3 millimetres in diameter, surrounded by indurated 
lung substance, the induration extending downward into the lung. 
The lung tissue in the lower lobe is hyperemia Pus can be 
squeezed from all the small bronchi, and there are small scattered 
foci of consolidation. On the upper border of the lower lobe is a 
small recent infarction. The surface of the lower lobe of the right 
lung is hyperemia In the upper lobe are numerous areas of con- 
solidation, the largest about 2 centimetres in diameter. In the 
lower lobe pus can be squeezed from all small bronchi. There are 
also numerous small foci of consolidation. The mucous membrane 
of the bronchi of both lungs is intensely hyperemia 

The right parotid gland presents extensive purulent infiltration. 
The external ear is intensely reddened and hyperemia 

Pharynx. — The mucous membrane of the pharynx is swollen and 
intensely hyperemia 

Spinal Cord. — The membranes and medulla are apparently 
normal. 

Microscopic examination of the fresh tissue by Dr. Councilman 
showed the heart muscle to be slightly degenerated (fatty), the 



28 CHOREA. 

epithelium of the kidneys swollen and granular, fatty in the upper 
collecting tubes. The liver was normal. Cultures were made from 
the blood in the right and left ventricles, and from the valvular 
vegetations, also from the parotid gland. Those from the right 
ventricle remained sterile, probably on account of overheating; 
from the others there was an abundant growth of the staphylococcus 
pyogenes aureus. 

Anatomical Diagnosis. — Acute endocarditis of mitral valve. 
Abscess of parotid. Catarrhal pneumonia of both lungs with 
bronchitis. Old tuberculosis of the left lung. 

This patient was febrile throughout. Unfortunately the tempera- 
ture sheet from December 24th to January 2nd was lost. On the 
morning of the 2nd in the rectum the thermometer registered 105 •2°. 
It fell on the morning of the 3rd to below normal ; rose throughout 
the 4th, and was 104 before her death. 

Chorea insaniens is thus defined by Bernt 1 : — " Chorea insaniens, 
quando affecti simul insaniee specie tentantur, variosque et nonun- 
quam truculentos, sibi inconsuetos mores, exhibent." 

A very large proportion of all the fatal cases of chorea have active 
delirium with fever. The subjects of the maniacal form are com- 
monly young females, between the ages of fifteen and twenty ; but 
children are also attacked, and one of the most rapidly fatal cases 
on record (No. 15/ Cook and Beale) in which death occurred in 
130 hours, was in a girl of nine years of age. The disease may be 
severe from the outset, but more commonly the case begins as one 
of ordinary chorea, and the special symptoms develop gradually. 
So predominant are the mental symptoms that cases are not in- 
frequently admitted to Asylums (Nos. 20 and 21). At the outset 
there may be hallucinations, which quickly give place to a chattering 
incoherency, less often a furious mania. It is to be noted that, as 
in the case just given, the movements may diminish greatly in 
intensity or cease altogether before death. Fever is an almost 
constant, but not an invariable, accompaniment. The temperature 
may be very high; in No. 18 of the fatal cases it reached ioy^", in 
No. 11, 107°, and in No. 16, 41 '5°. The duration of the fatal cases 
is rarely more than two weeks. Though a very pernicious form, the 
mortality is probably not so high as the literature would indicate, 
since there is a natural tendency to report only the more severe 

1 Opus cit., p. 21. 

2 These numbers refer to the tables of fatal cases in Appendix. 



MOTOR SYMPTOMS. 29 

cases. Gee lost only one out of seven ; and one of his patients 
with high fever and double parotitis recovered. 1 

Motor Symptoms. — The various muscle groups are not equally 
affected ; in a majority of the cases the hands are first involved, 
then the face, and subsequently the legs. The movements begin 
most often on the right side; thus of 410 cases analyzed with 
reference to this point, there were 126 cases in which they were at 
first dextral, starting usually in the hand, and only 80 instances in 
which the left side was first attacked. The movements rarely begin 
in the legs ; in only six instances out of the series of 410 cases was 
it so noted. In about a dozen instances the movements were said 
to have been general from the first, but the fact that early symptoms 
are readily overlooked must be taken into consideration. In 126 of 
the cases the movements became general. The muscles of the 
trunk and thighs are as a rule bilaterally affected. We rarely see 
the one group of shoulder muscles involved without the other, and 
the facial grimaces are not often confined to one side. 

Weir Mitchell has pointed out that there are differences in the 
character of the irregular movements ; thus, in one group com- 
prising the greater number of cases there was " awkwardness and 
inco-ordination of voluntary movements, followed soon or late by 
automatic or unwilled clonic spasms of various parts." In a second 
group the disease does not get beyond the state of inco-ordination, 
and irregular movements only occur during willed actions. In a 
third group there are " constant, automatic, irregular, clonic spasms, 
usually of the hands, but during volitional acts these entirely vanish, 
and the most complicated acts are well performed without obvious 
inco-ordination." In rare instances the movements become much 
less marked, or even cease when the patient stands up (Bernt). 

In nearly one-fourth of the cases the speech is affected. When 
slight, the disturbance may be limited to a slight impairment or 
hesitancy, and the child utters the words plainly ; in other instances 
speech is an incoherent jumble; the tongue and lips move, and 
sounds are uttered ; but it is impossible to recognize the words. 
Sometimes a few words are spluttered out in an explosive manner. 
The inability is in articulation rather than in phonation. The lips 
and tongue are more concerned than the intrinsic laryngeal muscles, 
or those of the expiratory group. In one case, however, with 
marked spasm of the inspiratory muscles and involvement of the 

1 Bernt says, p. 77, " Aegre etiam Chorea insaniens curam admittit." 



30 CHOREA. 

larynx, as shown by the whistling inspiration, the speech was not at 
all involved. In rare instances a difficulty in articulation is the first 
symptom noticed. As a rule, when the involuntary movements are 
of a very severe grade, the child will make no attempt to speak. It 
is to be remembered also that with very slight motor phenomena, 
and even without involvement of the facial muscles, the child may 
not talk at all, owing to psychical disturbances. 

As a rule the movements cease during sleep, but in some cases they 
persist. Possibly in some of these instances the mothers have mis- 
taken the irregular twitching, so often seen during sleep in nervous 
children, for the movements of chorea. In Case 1 2 1 of the Infirmary 
Series the movements are said to have been first seen while the child 
was asleep, and in Case 119 (b), in a very severe attack, the statement 
as to the persistence of the movements during sleep is very specific. 
Indeed I have known movements to occur in a choreic child, even 
while asleep, of such severity that it required the nurse to keep it in 
bed ; thus in Med., No. 97 (Johns Hopkins Hospital), Charles I., 
aged 12, admitted in a fourth attack of very great severity, it is 
noted that on three separate occasions during the night of the 27th 
of June he had severe attacks of muscular movement in his sleep, 
which were very violent,- and caused him to toss about. It is also 
noted that while asleep his arm and leg moved almost incessantly. 

Do the choreic movements extend to the muscles of organic life ? 
The great gastro-intestinal muscle is never involved ; there are 
certainly no symptoms which can be referred to irregular con- 
tractions of the coats of the stomach or bowels. The patients rarely 
complain of colicky pains. The sphincter ani is uninvolved, and 
the bladder acts normally as a rule, even in aggravated cases. Naturally 
there are a few instances of incontinence of urine, but not more than 
one Would expect in the records of any other disorder in children. 
Spasm of the muscle fibres of the bronchi, inducing asthmatic 
attacks, is not met with even in those cases in which the respiratory 
muscles are involved. The irregularity and rapid action of the 
heart, as well as the variability of the mitral systolic murmur, have 
been thought to be due to disturbed rhythm in the ventricular con- 
tractions, and to choreic spasm of the papillary muscles, but for this 
there is no very satisfactory evidence. The point will be referred to 
again under the cardiac symptoms of the disease. Braxton Hicks 
has reported a case 1 of chorea in pregnancy, in which during the 
attack the usual orderly contractions of the uterus became very 

1 Lancet, 1889, II. 



MUSCULAR WEAKNESS. 3 I 

irregular. The pupils may be dilated or insensible to light ; some- 
times they are unequal. 

Muscular Weakness is a not uncommon symptom. 

Sydenham spoke of the " unsteady movement of one of the legs 
which the patient drags." Dover and Mead both regarded chorea 
as a paralytic affection ; while Bouteille in the first case given in his 
monograph describes a paralytic enfeeblement of the affected side. 
Todd 1 has described very fully the paralytic form, to which West has 
given the name limp-chorea (chorea mollis). 

The condition is usually one of enfeeblement of the muscular 
strength, rarely of actual paralysis. The distribution of the weak- 
ness may be hemiplegic, paraplegic, or more frequently monoplegic. 
In twenty-eight of the Infirmary cases in which there was a special 
note of loss of power, the distribution was as follows : — paraplegic, 
six cases ; hemiplegic, four cases ; loss of power in left arm, ten 
cases ; in right arm, six cases ; in both arms and legs, one case ; 
in both arms, one case. The paralytic symptoms may precede, 
accompany, or follow the onset of the irregular movements. In 
the first group the diagnosis may present difficulties ; thus a girl 
was carried into my wards two years ago with the diagnosis of 
infantile spinal paralysis. In Case 89 (Infirmary Series) a girl, aged 
sixteen, with subacute rheumatism, had a sudden loss of power in 
the right arm and leg, with loss of voice, but without loss of 
consciousness. The attack lasted only half an hour, and was 
followed by pains in the limbs and choreic movements. The case 
was under observation for two months, and made a good recovery. 
In the majority of cases the weakness comes on during the attack, 
and is slight. The child may be unable to lift the arm to the head, 
the shoulder may droop, or the grasp may be feeble. Sometimes 
there is wrist-drop. Usually the weakness disappears with the 
cessation of the movements. Occasionally a local paralysis or 
weakness remains. In Case 229 (Infirmary Series) a lad of ten 
had severe general chorea in September 18S0, with loss of power 
in the legs. He recovered, but when he returned in September 
1883, in a second attack of chorea, he presented talipes of the left 
foot, the result of the paralysis in the former attack. In Case 21 
(Infirmary Series) wrist-drop persisted for two years, the result of a 
palsy which came on with chorea of the right arm. 

The monoplegic cases in young girls about the age of puberty may 
be difficult to separate from hysterical monoplegia until the choreic 
1 Medical Gazette. London, Vol. VIII, p. 849. 



32 CHOREA. 

movements develop, or the diagnosis may be complicated by the 
existence of other conditions, as a tuberculous adenitis in a case 
recently reported by Massalongo. 1 There may be at first, too, difficulty 
in determining whether a patient has chorea minor or is the subject 
of post-paralytic movements. In Case 9 1 (Infirmary Series), a girl, 
aged five, had when two years old congestion of the brain followed by 
a hemiplegia, which disappeared in a few days. Since the attack 
there had been more or less general choreiform movements, which 
when she came under observation were slight but distinct, and 
under treatment disappeared in three months. 

In severe cases of chorea with great jactitation the child may 
lose flesh rapidly and the muscles become thin and flabby. Occa- 
sionally in the affected limb the atrophy may be quite marked, and 
recently Raymond 2 has reported a case of marked monoplegic 
atrophy in the course of chorea. Perisson 3 has also written on the 
same subject. - 

1 Revue Netirologique, 1893, Vol. I. 

2 Sociite Midicale des Hopitaux, 1890. 

3 Bordeaux Thesis, 1890. 



33 



CHAPTER III. 

SYMPTOMS— {continued). 

Disturbances of Sensation — Headache — Mental Symptoms — Cutaneous Symp- 
toms — -Urine, Nephritis — Fever — Duration — Recurrence — Chorea in Preg- 
nancy — Chorea Minor in the aged. 

Disturbances of Sensation. — Sensory troubles play a very 
much less important part in the history of chorea. Pain in the 
muscles or nerves of the affected limbs is not very common, but 
occasionally instances are met with, usually of hemi-chorea, in 
which pain without arthritic complication is a marked symptom. 
Mitchell has spoken of these as cases of "painful chorea." Some 
writers have laid special stress upon painful points over the sites of 
emergence of the spinal nerves, and it has been stated further that 
in instances of hemi-chorea the pain is only on the side of the spine 
belonging to the affected half of the body. The nerve trunks 
themselves may present special tenderness. Among French writers 
Triboulet and Marie have described carefully these sensitive points. 
I muet say that in our experience at the Infirmary, and in my 
own personal experience subsequently, though I have frequently 
looked for these symptoms, they have been very rarely observed. 

Numbness, tingling, and pricking sensations are occasionally met 
with. Hemi-anaesthesia, which has been noted, is usually an 
hysterical feature. Anaesthesia of the pharynx has also been 
observed (Comby). 

Multiple neuritis occasionally occurs in chorea. Fry 1 has 
described the case of a girl aged 12, who had during a relapse of 
chorea increasing paresis, first of the lower extremities, and later 
also of the upper extremities, with atrophy and moderate con- 
tracture of the affected muscles, together with total anaesthesia, un- 
pleasant sensations in the fingers and toes, and loss of reflexes. The 
muscles of the head, neck and trunk were uninvolved. The patient 
ultimately recovered. Railton 2 has reported a case in which the 

1 Journal of Nervous and Mental Diseases, 1S90. 

2 Medical Chronicle, 1S86. 



34 CHOREA. 

paraplegia following chorea in a child aged 10 seemed due to 
neuritis. 

Epileptiform seizures may occur, and in the Infirmary Series 
there are records of five cases with convulsive attacks or periods 
of sudden unconsciousness. Case no had a severe convulsion 
during the course of the disease. In Case 410, a child, aged 9, had 
had for five years epileptiform attacks, three or four in each year. 
When seen in May 1887, she was in a second, very severe attack 
of chorea. In Cases 202, 232, and 248 there were attacks of loss 
of consciousness, probably epileptic in character. Gowers 1 has 
called attention to the relation of chorea to convulsive seizures, and 
has reported ten cases. 

Other forms of spasmodic disorders may coexist with chorea 
minor. Fry 2 has reported instances of athetoid movements, of 
rhythmical spasms, probably hysterical, and of tremor in cases of 
Sydenham's chorea. 

The condition of the reflexes has not often been studied. Sinkler, 
in fifty cases at the Infirmary, found the knee-jerk normal in 
twenty-six, diminished in fifteen, while in nine it could not be ob- 
tained. 

Trophic lesions are not common in chorea, unless we regard, 
as some would have us do, the joint troubles, as arthritis occurring 
in the course of a cerebro-spinal disease. 

Headache is a frequent, and in some cases a persistent, symptom. 
It may precede an attack for some days. It may be paroxysmal. 
The special senses are rarely involved. The question of eye-strain 
has already been fully considered. Among rare eye symptoms may 
be mentioned embolism of the retinal artery, of which cases have 
been reported by Benson, 3 Swanzy, 4 Sym 5 and Ball. 6 Minute 
retinal haemorrhages may also be due to embolism. Gowers states 
that in a few cases optic neuritis occurs, and he speaks of one case 
in which it was comparable to that seen in brain tumour. Gordon 
tells me that he has observed retinal hypersemia in many cases. 

Mental Symptoms.— Psychical disturbance is rarely absent in 
chorea ; fortunately in the majority of the cases it is slight in degree. 
The following are the most striking of the mental changes in the 

1 British Medical Journal, 1878. 

2 Journal of Nervous and Mental Diseases, 1892. 

3 Referred to in Lancet, January 30, 1886, p. 219. 

* Ibid. s Quoted by Gowers. 

6 Clinical Society's Transactions, Vol. XXI. 



• MENTAL SYMPTOMS. 35 

disease: (1.) Disturbances of the moral sense, manifested frequently 
in a strange perverseness, great irritability of temper, with emotional 
outbreaks. A frequent complaint heard from the mother is that the 
character of her child is completely changed. A patient may do 
odd and meaningless acts, thus, Case 150, a girl of 21, in a second 
attack had the trick of hiding away her clothes, and was in the 
habit of wandering off by herself. On one occasion she went off 
and was not found for two days. 

(2.) Disturbances in the faculties of memory and attention. The 
aptitude for study is lost, and the child may no longer take an 
interest in story books. This is of course very much more marked 
in some cases than in others. Transient loss of the power to read 
and to write may exist. In the extreme instances we can often 
"read the mind's complexion in the face," and can see, as the 
disease progresses, a cloudiness obscuring the bright, clear coun- 
tenance, leaving it dull, heavy, and expressionless. Actual melan- 
cholia may occur ; in rare instances the impairment of intellect may 
be progressive and terminate in dementia. Marce, whose essay 1 on 
the subject remains the most important, and Axenfeld and Huchard 2 
lay great stress on hallucinations by day and in the intervals between 
sleeping and waking. 

(3.) While the great majority of the cases of chorea minor display 
nothing more than slight mental changes, the rarer, more aggravated 
forms, coming under the head of chorea insaniens, present, as their 
most -striking characteristics, hallucinations, delusions, and mania. 
So striking may these features be, that there are many instances on 
record in which the bodily trouble has been entirely overlooked, 
and the patient has been committed to an asylum. 

Whether there is a chronic form of insanity deserving the name 
oifolie choreique is extremely doubtful. 

Cutaneous Symptoms. — Skin affections in chorea are not very 
uncommon. Some of them are due no doubt to the disease itself; 
others follow the prolonged administration of arsenic, which is so 
commonly employed in this disease. Out of 410 of the Infirmary 
cases there were eleven with skin affections. In five of these the 
rash was erythematous or papular, and unquestionably due to arsenic. 
There were two cases of herpes zoster. In Case 2, a girl of 7, the 
rash came out on the right side about three weeks after she began 

1 De Pe"tat mental dans la Chorie. Mimoires de P Academie Imperial de 
Medicine, XXIV. 

2 Traiti de Ndvroses. Second Edition, 1883. 

D 2 



36 CHOREA. 

to take arsenic. In Case 318, a girl of n, the herpes was on the 
left side in the lumbar region, and appeared seven weeks after the 
beginning of the use of arsenic. Pigmentary changes in the skin 
have been frequently noted in chorea, and are probably always a 
consequence of the prolonged administration of Fowler's solution. 
In Case 332, a boy aged 7, there were pigmentary changes on the 
right cheek after a six weeks' course of Fowler's solution. 

Gradual pigmentation from the excessive use of arsenic is quite 
well recognized, and many such cases are to be found in the 
literature. 

Among the most interesting cutaneous manifestations of chorea 
are those associated with arthritis, which are very similar to those 
developing in so-called rheumatic purpura. The most common 
are those which take the form of multiple erythema, either an 
erythema nodosum, a purpuric urticaria, or sometimes a simple 
purpura. In Case 13, a girl aged 18, without any rheumatic mani- 
festations, had an eruption resembling that of erythema nodosum, 
which persisted for some days. In Case 312, a girl aged 8, with 
pains in the wrists, at the time of the attack black and blue raised 
spots appeared on the legs ; they came out in crops and were not 
painful. They showed themselves before the administration of 
arsenic. In Case 347, a child aged 8, had a first attack of chorea in 
1885 and a second in 1886, the latter having been preceded by an 
attack of acute rheumatism. The knees and ankles were swollen, 
and there were large purple blotches on the skin of the legs and of 
the arms. 

In four of the recent fatal cases (see Morbid Anatomy) there 
were skin rashes in two resembling the scarlatinal eruption of septi- 
caemia, in one purpura, and in one there was the macular rash of 
secondary syphilis. 

Urine in Chorea. — An increase in the amount of urea was deter- 
mined by Walshe, Bence Jones, Kelly and others. Handheld Jones 
found the phosphates increased. Albuminuria is not uncommon 
in the very severe forms, and glycosuria has been described. Herter 
(private communication) found the uric acid much increased, and in 
several cases the chlorides were diminished. Todd, in his " Lectures 
on Diseases of the Nervous System," says that the uric acid may 
form a sediment, as in gouty conditions. 

A. E. Garrod 1 found uro-hsematoporphyrin present in the urine of 
fourteen out of twenty choreic cases. This substance is described 
1 Lancet, 1892, I. 



URINE IN CHOREA. 2>7 

by McMunn as particularly frequent in the urine of rheumatic per- 
sons, and Garrod looks upon this as an evidence of the close rela- 
tionship of the two diseases. 

Nephritis is probably not so rare as has been thought. Thomas, 1 
of Freiburg, has reported a case of a boy of 14, who had not 
previously had either chorea or nephritis, and who, three weeks after 
the first outbreak of chorea, had general anasarca and the signs of 
acute nephritis. Prof. Thomas thinks there was an etiological con- 
nection between the two. 

In Case 6 of the Guy's Hospital series reported by Goodall, 2 a 
girl aged 17, who had never had rheumatism, was admitted 
in her second attack of chorea. Pericarditis was detected. She 
died suddenly and unexpectedly. There was slight pericarditis, and 
recent endocarditis of aortic and mitral valves. The kidneys weighed 
13 ounces, were enlarged, red, with swollen cortices, and showed all 
the signs of an acute nephritis. 

In one of Turner's cases (No. 60 of series of fatal cases), a girl of 
17 had acute nephritis for a week, with haematuria, and No. 12, a 
girl of 1 1 years, also had hsematuria. 

Fever in Chorea. — Except in the severe maniacal form, fever is 
rare. In the Collective Investigation Report of the British Medical 
Association pyrexia was stated as being present in only 12 per cent. 
The study of the disease in out-patients does not favour thermo- 
metric investigation. Of twenty-eight cases treated in my wards at 
the Johns Hopkins Hospital there was only one, a case of chorea 
insaniens, in which the temperature rose to 105 '2°. In eleven 
instances the temperature registered above ioo° ; in six of these 
above 101°. As a rule, anything more than a slight rise of tempera- 
ture is usually to be referred to a complication ; thus, Jos. C, 
Medical No. 3,064, had been ill for two weeks with pain and swell- 
ing of the feet, and chorea. When admitted, July 3rd, 1893, he 
had a temperature of 104 . The choreic movements were active. 
There was a soft systolic murmur. Under the salicylates the tem- 
perature fell, was normal on the 6th, and remained so. In other 
cases the fever seems due to an endocarditis, or to an endo-peri- 
carditis. Occasionally one meets with cases in which it persists for 
days without any obvious cause except the chorea itself ; thus, in 
the case of Marion S., admitted July nth, 1892, Medical No. 2,191, 
the patient had had chorea for several weeks without any articular 

1 Neurologisches Centralblatt, Bd. XI., p. 391. 

2 Guy's Hospital Reports, Vol. XLVII. 



38 CHOREA. 

affection and without any heart symptoms. The temperature from 
the nth to the 18th ranged from 99'5° to nearly 101°. A still 
more striking case is the following : — Laura D., aged 14, Medical 
No. 1,527, admitted September 3rd, 1891, in her third attack of 
chorea, which had lasted for several weeks. She had not had 
articular trouble, but her father has had rheumatism. On admission 
she had chorea of moderate severity ; the heart was not affected. 
The temperature was 99*3° ; on the 6th and 10th it rose to 
100°. She complained frequently of being chilly. On the 12th 
the temperature rose to 103 , and in the evening nearly to 
105 . There was no joint affection, and the throat was not sore. 
The temperature kept up, and for the next three days was frequently 
in the neighbourhood of 104 . Then from the 15th to the 18th it 
ranged from 100° to 102 . On the 19th it became normal. We 
were at a loss to know exactly upon what this fever depended. 
There seemed to be slight enlargement of the spleen ; the choreic 
movements persisted, but there was no sore throat and no heart 
complication. It was suggested by Dr. Thayer that possibly this 
might be an illustration of Kahler's non-articular rheumatic fever, 1 
and on the 18th she was ordered sodium salicylate, five grains every 
two hours. The temperature at the time was 102 . At 4 a.m. on 
the 19th it was 987°, the lowest point reached since entrance. At 
6 p.m. on the 19th it was 10 1°, and fell to 99° in the evening. The 
salicylates were kept up for three days, and she had no more fever 
until November 8th and 9th, when she had a rise of temperature to 
nearly 103°. She was discharged well on November 23rd. 

Finlayson 2 (Glasgow) has written an interesting paper upon short 
febrile attacks in chorea, which he suggests are due to the endo- 
carditis. He gives the case of a child aged 7, admitted to hospital 
November 18th, 1884, with a chorea of the left side of seven weeks' 
duration. There was no history of rheumatism. There was an 
apex systolic murmur. The chorea improved, but on December 14th 
the child began to have fever, the temperature rising to 103 without 
any local manifestations. There was variable fever throughout 
January, in February it increased, and the range was from 102° in 
the morning to 105 in the evening. Post-mortem there was found 
acute endocarditis of the mitral and tricuspid segments, with very 
slight fresh pericarditis. It is important to bear in mind that the 
fever may also be kept up by pericarditis, as in the case reported 

1 Zeitschrift f. Klin. Medicin, Bd. XIX. 

2 Archives of Pediatrics, Vol. VII. p. 97. 



FEVER IN CHOREA. 39 

by Sinkler, 1 of which I give an abstract in the section on the 
heart. 

The temperature may be increased on the affected side. H. A. 
Hare 2 has reported several such cases ; one of his patients said 
that he felt the hand getting hot, as the movements became exag- 
gerated in the limb. 

In cases of maniacal chorea, and in the severer forms without 
delirium, fever is usually present, and may reach a high grade. In 
one of Mitchinson's cases (No. 18) the temperature was ioyd , 
In a case reported by Grosse (No. 16) it was 41 "5° C, and in No. 11 
(Donkin and Hebb) the thermometer registered 107 . There 
may be, however, the most severe chorea with complications, pneu- 
monia and pericarditis, without any fever to speak of, as in the 
case of Neuwerck (No. 66). 

Duration. — Very mild cases may terminate in three weeks, but 
from eight to ten weeks may be stated to be the average duration of 
attacks of moderate severity. Cases, however, very frequently drag 
on for three or four months, and there are instances of true chorea 
minor in which the movements persist for even six months. The 
term acute chorea may very properly be applied to the very severe 
cases with fever and delirium, which may terminate within a week or 
two after the onset. The very chronic cases in children and in 
young adults are very rarely true chorea minor, but belong to a 
different category, and are usually forms of tic. The instances of 
congenital chorea and chorea spastica have nothing to do with the 
form at present under consideration. The following cases, however, 
may be instances of unusually protracted chorea minor : — 

Case 186, Infirmary Series, Lizzie K., aged 21, no history of 
rheumatism. When thirteen years old she had the first attack, 
which was general and affected the speech. After lasting without 
intermission for three years the movements ceased, and she returned, 
December 1st, 1882, with a second attack, which had already lasted 
three months. 

Case 335, Infirmary Series, Mary M., aged 12. Mother very 
nervous, and an uncle had spasms. For three years she had had 
movements in the face, head and arms. The speech was never 
involved. On September 21st, 1885, when first seen, the head was 
chiefly affected. Jerking movements of the hand also occurred 
when at rest. She was treated for some months without any im- 

1 University Medical Magazine, 1S90, p. 483. 

2 Boston Medical and Surgical Journal, Vol. CXII. 



40 CHOREA. 

provement. On the 7th of May, 1887, she returned to the clinic. 
The choreic movements had gradually disappeared, and though 
nervous she seemed quite well. 

The following is a very remarkable case, which was under 
observation for some years : — 

Alfonso G., aged 21, baker. His sister had chorea, and died of 
heart disease in 1872. In August, 1884, he began to have jerking 
movements in the head and arms and legs. The speech was also 
somewhat affected. When seen June 1st, 1885, there was spasmodic 
contraction of all the muscles of the face, neck, trunk and arms. 
The legs were not affected. The inspiratory muscles were involved, 
and at times there was high-pitched whistling inspiration. The 
heart was not affected. He remained under observation and treat- 
ment for nearly three years, during which time the movements 
persisted with very little variation in intensity. When I last saw 
him the twitching and jerking of the muscles of the neck and chest 
were present, and the case then looked more like an instance of 
habit spasm. 

It is quite possible that the localized movements of a true chorea 
minor may persist as facial tic, and Leroux 1 has reported cases of 
this post-choreic tic, but they are of extreme rarity. 

Recurrence. — The liability of chorea to recur was noticed by 
Sydenham, who says that " as the disease is liable to return again, I 
think it well for the patient to be purged about the same time, or a 
little earlier, the following year." In the 410 cases analyzed 
with reference to this point, in 240 there was a single attack ; 
in no, two; in 35, three; in 10, four; in 12, five; and in 
3, six. Case 23 of the Infirmary Series is a good illustration. 
A girl, aged 8, came to the clinic for the first time March 23rd, 1877. 
The attack had begun in the autumn of the previous year. She 
improved under treatment, and the movements ceased. Towards 
the end of June the general symptoms returned, and this is noted as 
the second attack, though it probably was only a relapse. The 
third attack was in August, 1880, the fourth in the spring of 1881, 
the fifth in January, 1882, and in February, 1883, she returned with 
a sixth attack. There was no rheumatism, and the heart was not 
affected. Gowers mentions a case in which there were nine attacks. 

We know very little as to the cause of the recurrences. Females 
appear to be more liable to them than males, but this is only natural. 
It is doubtful whether rheumatism has any influence. In cases with 
1 Revue des Maladies de V Enfance, 1891. 



FEVER IN CHOREA. 4 1 

frequent recurrence heart disease is more common ; thus, of the 
seventy-two cases to be hereafter noted with organic disease follow- 
ing chorea, thirty had had three or more attacks of chorea. 
Mitchell has called special attention to the vernal recurrence of 
chorea, and at every clinic there are many instances in which during 
the months of March, April, and May, children are brought back 
with fresh attacks of the disease. It was a custom at the Infirm- 
ary for Diseases of the Nervous System to warn the parents 
on this point, and give instructions to be particularly careful, and 
even to resume the arsenic in the spring. The recurrence may, 
however, be autumnal. 

The interval between the attacks is usually many months, or it 
may be several years. In a majority of the cases as long a period 
as twelve months intervenes. Fright and over- work at school are 
often mentioned as exciting causes of the recurrence. The succeed- 
ing attack may be much more severe than the first. The same 
muscles are usually involved in the recurrence as were affected in 
the primary attack, but this is not always the case. Other muscles 
may be involved, and though the recurrence is apt to be of less 
intensity, there are instances in which it is very much more severe. 
The heart may not be affected until after the third attack, and as in 
the case above mentioned, even a series of attacks may occur 
throughout several years without any cardiac involvement. 

CHOREA AND PREGNANCY. 

The association was noticed by several observers during the last 
century. Important statistical papers have been written by Barnes, 
Mosler, and others. 

The following cases have occurred at the Infirmary : — 

Case 277. — A woman, aged 20, who had had repeated attacks of 
chorea, applied at the clinic October 15th, 1884. She was married 
early in May. Began to have movements of limbs August 15th. 
At time of visit both sides were involved, and there was decided 
affection of speech. She had morning sickness, the menses had 
stopped, the breasts were enlarging, the papillae prominent, and she 
was believed to be pregnant. She attended at the clinic for four 
weeks, and improved very much. The mental condition was good. 
There is no note as to her complete recovery. 

Case 236. — Mrs. B., aged 22, applied at the clinic November 16th, 
1883. She had an attack of chorea when nine years of age, and 
has been nervous ever since. Is pregnant. Attack began about 



42 CHOREA. 

September 1 5th, and has recently got worse. The right side chiefly 
involved ; speech much affected ; no mental symptoms. She 
remained under treatment for a few weeks, improved slightly, but 
did not return. 

A third case was admitted to the Hospital in the spring of 1876. 
The notes have been mislaid, and I am indebted to Dr. Neff for the 
following memoranda. She was aged about 30, a servant at the 
Colonnade Hotel. She had had chorea as a child. In the fourth 
or fifth month of pregnancy she accidentally broke a demijohn of 
brandy, and received a severe shock. Soon after chorea developed, 
and she was brought to the hospital. The attack was very severe, 
and after two months she was transferred to the maternity depart- 
ment of the Philadelphia Hospital, where she died. 

A fourth case was under treatment by Dr. Sinkler in 1887. 
Woman aged 21, married three months. Menses had stopped and 
she was believed to be pregnant. Nine years ago she had chorea — 
a most obstinate attack — which persisted for five years without inter- 
mission. She had a second attack, which lasted two months. The 
present one began shortly after her marriage, and is, possibly, not 
directly dependent upon pregnancy. It is, so far, not a serious 
attack. 

The following case came under my observation at the University 
Hospital : — 

Jennie C, aged 18, seen on the 4th of June in the Medical 
Dispensary of the University Hospital. 

She was married last March. Healthy and well as a child, and 
as a young girl ; has not menstruated since marriage ; believes that 
she is pregnant. A week ago she noticed jerky movements of the 
left arm, the left leg, and the left side of her face : had been feeling 
nervous sometime before. She is somewhat anaemic. 

She now has distinct hemi-chorea with irregular movements of 
the hand and arm, but less marked movements of the foot and leg ; 
the left side of the face twitches ; when the tongue is protruded it 
moves about irregularly ; she complains of loss of power in the left 
arm ; pulse 96 ; temperature not elevated. 

The apex beat of the heart is in normal position; there is 
soft systolic murmur obliterating the first sound; it is not heard 
at mid-axilla. In the fifth interspace toward the sternum both 
sounds are heard with distinctness ; in the second, third, and fourth 
left interspaces there is a loud systolic murmur with marked accen- 
tuation of the second sound ; no murmur at the aortic cartilage. 



CHOREA AND PREGNANCY. 43 

Subsequently this case came under the care of Dr. Wood and 
Dr. Hirst, the latter of whom has reported it. 1 The history is of 
interest, as it appears that her unilateral chorea improved very much 
but she became profoundly apathetic and melancholy, and had very 
pronounced anaemia. The choreic movements stopped entirely. 
So serious, however, was her mental condition that it was thought 
advisable to bring on premature delivery, which was done success- 
fully. The prolonged apathy and melancholy is seen also in children. 
In a case which was in my wards last winter the condition persisted 
for more than three months. 

Primiparae are most frequently attacked, and a previous chorea 
plays an important role ; thus out of thirty-four recent cases collected 
by McCann, 2 eleven had had chorea previously. In his statistics 
he does not give the number in which it occurred during the first 
pregnane)^. Fright, mental emotion, and anaemia are mentioned as 
special factors. 

In his paper he gives the following statistical details : — In thirty- 
seven cases the ages were as follows : — At seventeen, 3 ; at eighteen, 
4 ; at nineteen, 6 ; at twenty, 1 1 ; at twenty-one, 2 ; at twenty-two, 
3 ; at twenty-three, 3 ; at twenty-four, 1 ; at twenty-five, 1 ; at twenty- 
six, 3. 

The period of occurrence during pregnancy was as follows : — ■ 
" Out of 36 cases, first month, 2 ; second month, 6 ; two-and-a-half 
mpnths, 1 ; third month, 6 ; three-and-a-half months, 2 ; fourth 
month, 7 ; fifth month, 4 ; five-and-a-half months, 2 ; sixth month, 
3 ; six-and-a-half months, 1 ; seventh month, 1 ■ eighth month, o ; 
ninth month, 1." 

The following details as to causation are given : — " Out of 34 
cases, chorea previously, 1 1 ; rheumatic and scarlet fevers, 2 ; 
rheumatic fever alone, 2 ; fright only cause stated, 2 ; no cause 
stated, 4 (in one of the cases one sister had had rheumatic fever ; in 
another chorea and rheumatism existed in the family) ; rheumatic 
fever and fright, 1 ; rheumatic fever and chorea, 7 ; scarlet fever, 
rheumatic fever, and chorea, 1 ; chorea and fright, 3 ; mental 
distress owing to pregnancy, 1." 

Out of 32 cases in which there was no artificial interference the 
patient was delivered at term in 26. In one case there was 
accidental haemorrhage and miscarriage at the fourth month ; in one 
case miscarriage at the fifth month ; and in four cases miscarriage at 

1 University Medical Magazine, Philadelphia, Vol. I. 

2 Transactions of the Obstetrical Society of London, Vol. XXXIII. 



44 CHOREA. 

the sixth month. Out of 39 cases death occurred in 7 ; in 3 from 
mania ; in 3 from exhaustion ; and in 1 from puerperal peritonitis. 

In the 72 fatal cases which I have collected from the literature 
since 1881 only four cases were associated with pregnancy. This is 
a very much smaller percentage than in Raymond's tables, in which 
of 79 fatal cases 11 were in pregnant women. 

Post-partum chorea is much more rare. McCann refers to only 
three or four cases, but he states that it is not yet definitely settled if 
this form of the disease exists. The following instance, reported by 
Litten, 1 seems to be genuine : — 

A primipara was delivered on December 3rd of a living child. On 
the sixth day after delivery she was sent to the medical clinic with 
an extensive scarlatinal rash and high fever. On the third day after 
admission the rash faded, but the fever kept up. On the evening 
of December 9th she had a chill, and many of the joints became 
swollen and painful. The lochia were offensive. On the 13th of 
December choreic movements began in muscles of limbs, which 
became general and severe. There was irregular fever, which with 
the arthritis made up a picture of puerperal fever. Death on 
December 19th. Autopsy : Diphtheritic endometritis ; polyarthritis ; 
purulent septic infarcts ; heart normal. 

It is to be borne in mind that hysterical disorders of motion may 
occur post-partum. 

CHOREA MINOR IN THE ADULT AND IN THE AGED. 

The comparative rarity of the disease in the adult is well shown in 
the statistical table, which gives in the third decade only ten cases ; 
in the fourth decade only one ; and above the fourth only two. 
Sinkler 2 has reported two cases in patients over eighty. Saundby 3 
has collected twelve cases. Many of the cases reported as chorea 
belong in reality to affections quite different from chorea minor. 

Herringham, 4 who has given a critical analysis of the cases 
described as chorea in the aged, divides them into four groups : first, 
chorea minor; second, choreiform movements due to coarse sub- 
cortical lesions ; third, the chronic progressive chorea of Hunting- 
ton ; and fourth, a chronic chorea of adults and the aged, without 
hereditary bias as in the Huntington's form, but with coarse 
cortical changes, and accompanied by mania and dementia. The 

1 Charite 1 Annalen, Bd. XI. p. 279. 

2 Journal of Nervous and Mental Diseases, July, 1 88 1. 

3 Lancet, 1884, II. 4 Brain, Vol. XI, 1889. 



CHOREA MINOR IN THE ADULT AND AGED. 45 

following is a good illustrative case of Sydenham's chorea in an old 
woman : — 

Lousia B., mulatto, aged about seventy, was admitted December 
2nd, 1893, with choreic movements in the hands and arms, par- 
ticularly on the left side. The patient was a widow, and had had nine 
children, all of whom are dead. She was a domestic in the family 
of a lady who had promised to remember her in her will, but, failing 
to do so, the patient was very much worried and distressed, and 
about four or five weeks before entrance she began to have 
twitchings, and it was stated by the friend who brought her that she 
had not been quite right in her head. It was impossible to get a 
satisfactory history as to the date of the onset, but she was positive 
that she had never had any similar trouble before, and of the 
association of the attack with disappointment and the loss of her 
mistress. 

On admission her temperature was normal ; she was somewhat 
irrational ; could not be kept quiet, and would not stay in bed. 
She talked in an irrational manner, though she evidently understood 
what was said to her. She walked fairly well ; the arms and hands 
displayed quick movements, and there were occasional 1 jerkings of 
the muscles of the face. The muscular power was good. There 
was a loud, blowing systolic murmur heard over the whole cardiac 
area, but the heart did not appear to be hypertrophied. The urine 
was normal. The patient was ordered to be kept in bed and have 
Fowler's solution of arsenic, three minims three times a day. 

Throughout December she improved a good deal and the move- 
ments were very much less, being confined chiefly to the left arm 
and hand, and the left side of the face. Early in January they 
increased somewhat. She was able, though, to be up and about, 
and her mental condition seemed somewhat better, though she was 
rather stupid and dull. 

Throughout January and February she improved a good deal, but 
the movements never entirely ceased. She was able to be up and 
about the ward. 

In March the condition was unchanged. The movements were 
very slight, being confined chiefly to the left side of the face, and 
to the left hand and arm. Sometimes they were scarcely noticeable. 
Early in April she had one or two attacks of cardiac dyspnoea and 
had cough. On the evening of the 7th she had a very severe 
attack, in which she sat up, gasped for breath, became quite pulse- 
less, and died in a few hours. 



46 CHOREA. 

Autopsy. — There were no coarse cerebral lesions. The heart was 
hypertrophied, the mitral segments were thickened and insufficient 
from old sclerotic endocarditis ; there were no fresh vegetations. 

Herringham 1 gives the following abstracts of chorea minor in the 
aged and adult, which I here add : " Roger has recorded a case in 
which an old lady of 83 was affected by an ordinary chorea which 
came on without cause, and was cured in five weeks ; Russell writes 
of a woman of 77, who had it mainly on the left side, and recovered 
in three months ; Sinkler gives a case of a man of 86 who recovered 
in a few months ; Saundby gives two cases, but the histories do not 
extend for a sufficient length of time to enable them to be classified. 
Ferguson had a case of a woman of 74, who after much fatigue in 
nursing her husband, became somewhat irritable and excitable, and 
then began to twitch on the left side. She recovered in eleven 
weeks. In the Collective Investigation Report, there are several 
cases which Dr. Mackenzie has grouped together as chorea at ex- 
ceptional ages. No. 119, by Gowers, is a second attack of chorea 
following rheumatic fever in a man of 40. No. 223, by Alexander, 
is a woman of 63, in whom the attack lasted four months. She also 
had had it before. No. 222, by the same author, is a first attack in 
a woman of 68, lasting six months. Carline records a case, No. 180, 
of an old woman of 73, in whom a chorea lasted four weeks. She 
had had several attacks of late years. Aitken describes another 
case, No. 93, of a woman of 86, lasting one year. Of this kind also 
is Graves's case of a man of 70, in whom the attack lasted several 
months, and Gauthier records an attack in a woman of 75, due to 
peripheral irritation from extraction of a tooth, similar to those occa- 
sionally met with in the young. She recovered after fourteen days." 

The cases must be distinguished carefully from generalized forms 
of tic, and from the disordered movements associated with organic 
lesions. There is less likelihood of confusion with Huntington's 
chorea. The disease may prove of great severity, and run a malig- 
nant course. One of Koch's cases (No. 50 in the table) had had 
several previous attacks. Fresh endocarditis was found. No. 17, a 
man aged 66, had had chorea eight years before, and died in coma 
on the fourth day after admission, possibly from the effects of bromide 
of potassium and chloral. Fresh vegetations were present on the mitral 
segments. In No. 71 the chorea followed emotional disturbance in 
an old woman with mitral insufficiency. Death occurred in an 
attack of cardiac dyspnoea. There was no fresh endocarditis. 

1 Loc. cit. 



47 



CHAPTER IV. 

THE HEART IN CHOREA MINOR. 

Condition of the Heart during the Attack — Condition of the Heart in Eatal Cases 
— Subsequent Heart History in Choreic Patients — Pericarditis in Chorea. 

Rarely a fatal disease, a series of several hundred cases of chorea 
may be treated without a death j nor does it often leave permanent 
damage of the nervous system, since, so far as intelligence and 
motor power are concerned, the children may recover perfectly. 
The only serious event is the occurrence of endocarditis, and the 
danger here, not immediate but remote, lies in the changes which 
may be initiated by the acute valvulitis. A study of the cardiac 
relations of chorea must embrace (i) the condition of the heart 
during the attack; (2) its condition in fatal cases; (3) the sub- 
sequent heart history of persons who have recovered from the 
disease ; and (4) the state of the pericardium. 

I.— Condition of the Heart during the Attack. 

The bare chest should be examined. Auscultation through the 
clothing is not trustworthy, since soft murmurs, readily audible with 
the stethoscope placed directly against the chest wall, may escape 
detection. It is a good plan to let the child remain quietly on a 
lounge for some time, and make the examination first in the recum- 
bent posture. Subsequently the effect of exercise and of the erect 
position may be tested. In the severer forms of chorea the ex- 
treme jactitation may prevent for some days a satisfactory examina- 
tion. Children with chorea rarely complain of palpitation, pain 
about the heart, or of symptoms which would direct attention to the 
organ. As in acute rheumatism, it is for us to look for evidences of 
disturbed action. 

Anomalies in the cardiac action may depend upon faulty inerva- 
tion, upon changes in the muscular walls, or in the condition of the 
circulating blood, and upon inflammation of the valve segments. 

Palpitation and disturbance of rhythm, rapid action, and pain are 



48 CHOREA. 

the chief nervous manifestations. Palpitation and irregularity are 
not very common, and are not so frequent as the rapid action under 
emotional disturbance. Occasionally the heart-sounds have a foetal 
rhythm. Cases with very • exaggerated movements may present for 
days an excessively rapid heart action. On the other hand, with the 
mental enfeeblement which sometimes follows chorea, the pulse may 
be abnormally slow, beating in a child of ten or twelve at the rate of 
seventy or eighty a minute. I have never seen a case in which the 
disordered movement was of such a kind that it might be attributed 
to a special choreic action of the heart muscle {choree du cozier). 

The subjects of chorea rarely complain of pain about the heart, 
and even with very rapid action and palpitation there may be no 
subjective sensations. Pain as a marked symptom is met with in 
recurring attacks associated with endo-pericarditis and rheumatism, 
more rarely in the first attack with endocarditis. 

Evidence of involvement of the myocardium, of the valves, and 
of disturbed function due to impoverished blood is afforded by the 
presence at one or other of the so-called cardiac regions of the 
abnormal sound known as a murmur, the nature of which in chorea 
has been so much discussed. 

It may be well to speak first of the incidence of heart murmurs 
during the attack. Of the 554 cases at the Infirmary for Diseases 
of the Nervous System, 170, 307 per cent, presented heart mur- 
murs; in 149 apical in maximum intensity; in 21 basic. In 141 
cases of chorea minor examined at the Dispensary for Nervous 
Diseases of the Johns Hopkins Hospital, there were 42 with a cardiac 
murmur. Of the 449 cases in the Report of Committee on Collec- 
tive Investigation of the British Medical Association, 113 had heart 
murmurs. 

Much has been written in explanation of the occurrence of heart 
murmurs in this disease, and an excellent summary is to be found 
in Hayden's 1 work. 

The murmurs may be either functional or organic. 

Functional. — The basic systolic murmur heard usually with 
greatest intensity in the area of the pulmonary artery, but audible 
sometimes in the aortic area, may be due to the excited and very 
rapid action of the heart. A murmur of similar character is common 
in neurasthenic women, and may be heard in thin-chested subjects 
after violent exercise, and in children with the rapidly acting heart 
of high fever. I have had many opportunities of noting, after rest 

1 Diseases of the Heart and Aorta, 1875, Part I, pages 265-277. 



CONDITION OF HEART DURING ATTACK. 49 

in bed for a day or two, the disappearance of a systolic murmur at 
the base, which had been heard with great clearness during the 
excitement of the first examination of the child in the Dispensary. 

With anaemia and debility, frequent associates of a chorea in its 
third and fourth weeks, there may develop soft systolic murmurs in 
the pulmonary artery and apex areas, often, too, heard intensely over 
the body of the heart along the left sternal margin. Frequently 
with it one notices a wide area and fulness of cardiac impulse, and 
sometimes systolic pulsation in the cervical veins. The murmurs 
may be audible only in the recumbent position, disappearing when 
the patient stands up. These murmurs are in all probability caused 
at the pulmonary and tricuspid orifices, and are accompaniments of 
debility and anaemia. 1 In protracted cases with marked debility, 
and weakness of the heart muscle, the systolic apex murmur may be 
mitral in origin and be due to muscular insufficiency. 

Another explanation of the frequency of heart murmurs in chorea 
is the theory " that irregular and occasional reflux takes place at the 
mitral orifice through disordered action of the muscular apparatus 
connected with the valve " (Walshe). The objection to this view 
which Kirkes urged years ago, that there was no proof of the parti- 
cipation of involuntary muscular organs in the choreic disorder, still 
holds, nor is there such inconstancy and . variability in the apex 
heart murmurs of chorea as would be inevitable did the condition 
result from valvular insufficiency in consequence of a " want of 
correspondence between the fibres of the ventricle which obliterate 
the cavity and those that close the valve." 

There is experimental evidence to show that one set of the papil- 
lary muscles may be completely functionless without causing any 
murmur. Dr. Townsend Porter, of the Harvard Physiological Labora- 
tory, has shown that ligation of the ramus descendens of the left 
coronary artery causes within twenty-four hours complete infarction 
of the anterior papillary muscles from which the chordae tendineae 
pass to one-half of each mitral flap. On auscultation at a time when 
infarction was fully developed no murmur was noted on repeated 
auscultation in several animals. 

In a large proportion of all cases of chorea in which a murmur is 
heard at the base, or along the left margin of the sternum in the 
second, third and fourth interspaces, the disturbance is probably 
functional. 

1 See discussion of their causation in Investigations into some Morbid Cardiac 
Conditions. William Russell, M.D., Edinburgh, 18S6. 

E 



50 CHOREA. 

Murmurs of Organic Origin. — Acute endocarditis, commonly 
of the mitral leaflets, occurs with great frequency in chorea, and the 
remarkable statement that there is no other disease, not even acute 
rheumatism, which is so frequently accompanied with valvulitis, 
seems quite justifiable. The apex systolic murmur heard in many 
cases of chorea is doubtless due to the endocarditis. The symptoms 
and physical signs of acute endocarditis are very uncertain ; the 
mitral segments may be inflamed, and yet the patient may present 
no cardiac symptoms whatever. There are very carefully observed 
cases of chorea, in which the apex murmur has not been present, 
and yet post-mortem the mitral segments have shown vegetations. 
Feelings of oppression about the heart, palpitation, transient dys- 
pnoea, though mentioned as symptoms of acute endocarditis, are, 
after all, rarely present in the first attack, and are much more 
commonly seen in a mgjI^S^efi2B5»4itis attacking a heart already 
damaged. Fever,^roS> is a vanaBfe'^TOOtom, and is not always 

present. /{fcT V\ 

The physical signs, t^yi^iiina Jft&gpore reliable, are nevertheless 
inconstant. TheYapejc beat may be a httle /diffuse and the area of 
dulness increased, Xra^j^rlo^^n^b.di^g^ue to slight dilatation of 
the left ventricle. ArN^te^ionvo^itre first sound, which has a 
prolonged or dull character, with the subsequent occurrence of a 
blowing murmur at the apex region, developing under observation 
in a case of chorea, rheumatism or fever, and more particularly in 
the earlier stages, before the patient has become much enfeebled 
and anaemic, are the most reliable auscultatory signs. 

The following statements may be made on this question : — 

i. The extraordinary frequency with which mitral valvulitis is met 
with in fatal cases is remarkable. There is no known disease in 
which endocarditis is so constantly found, post-mortem, as chorea ; it is 
exceptional to find the heart healthy. 

2. The character and location of the apex murmur are such as 
experience in other affections has taught us to be associated with 
inflammation of the mitral segments. Why this murmur should be 
so generally connected with the presence of a row of small warty 
vegetations just within the auricular margins of the curtains, and not 
capable, as one would think, of seriously interfering with their 
functions, is a problem to be solved. The condition certainly does 
not necessitate regurgitation, and the bruit may perhaps, as has 
been suggested, be due to friction of the roughened faces of the 
segments. 



CONDITION OF HEART DURING ATTACK. 5 1 

3. The inconstancy of the murmur and its disappearance on the 
subsidence of the chorea have been urged against this view. As 
already stated the bruit may be variable, and, indeed, does not 
necessarily accompany mitral endocarditis. Kirkes, years ago, 
insisted upon this, and there have been two autopsies in carefully 
studied cases of chorea in which the vegetations were found post- 
mortem, although careful examination during life had failed to reveal 
a murmur (Baxter : Brain, Vol. II. ; Frank. Allg. Wiener Med. 
Zeitung, 1879). There are facts which suggest that we may during 
the attack have an endocarditis, not manifested even by a murmur, 
which has nevertheless laid the foundation of future trouble. The 
disappearance of the apex murmur of chorea — and of rheumatism 
also —has been repeatedly followed ; and if it is true that the murmur 
is caused by the small vegetations, that it should disappear is only a 
natural sequence of the changes which go on in them. At first a 
soft granulation tissue, they become in time firmer and smaller, until 
ultimately smooth flat elevations mark the spots. It is not improb- 
able that if we could follow accurately the auscultatory history of a 
valve affected with acute endocarditis, we should find in many cases 
that the murmur of the fresh attack disappeared, to reappear when 
the changes, which it is the misfortune of the acute disease to 
initiate, have reached a point at which they begin to interfere with 
the competency of the valve. 

4v In its sequel the cardiac affection of chorea has been supposed 
to differ from that of other diseases, " as none of the injurious after- 
consequences which attend endocarditis in its other relations, .... 
are found to ensue here " (Sturges). The examination of a large 
number of choreics some years subsequent to the attack tells a sad 
tale to the contrary, and proves that the primary heart trouble is, in 
a majority of cases, at least, an endocarditis. 

II. — Condition of the Heart in Fatal Cases. 

The statistics of fatal cases of chorea have been collected by 
Sturges 1 and Raymond. 2 Sturges states that of eighty cases repre- 
senting the combined experience of Guy's, St. Bartholomew's, St. 
George's, and St. Thomas's Hospitals, there were only five in which 
the heart valves and pericardium were reported healthy. In Ray- 
mond's table of seventy-nine cases, if we exclude the London cases, 

1 Chorea. London, 1881. 

2 Dictionnaire Encyclope'dique des Sciences Mcdicales. I Serie, 25. Art. 
Danse de Saint Guy. 

E 2 



52 CHOREA. 

thirty-four are eft ; in only nineteen of these are specific statements 
given as to the condition of the heart, and in every one of the 
nineteen endocarditis was present. I have collected from recent 
literature seventy-three additional cases, of which sixty-two presented 
endocarditis. The table of these cases will be found under an 
appendix to the section on " Morbid Anatomy." My personal experi- 
ence includes five cases, in four of which endocarditis was present. 
Brief notes of the cases are as follows : — 

Case I. — S., girl, aged n,had had acute rheumatism. Admitted 
to the Montreal General Hospital, under Dr. George Ross, with 
acute chorea, and died of an intercurrent pneumonia. The move- 
ments had almost ceased under hypodermics of arsenic. The 
autopsy showed slight hypertrophy of the heart, somewhat thickened 
mitral curtains, with numerous, irregular, warty vegetations just inside 
the auricular margins. Two of the aortic segments also presented 
bead-like vegetations below the corpora Arantii. 

Case II — T. B., a boy, aged n ; had chorea in May, 1880, and 
a second severe attack in July of the same year. No rheumatism. 
No heart murmur. About the 20th of February, 1881, there was a 
recurrence, and on March 3rd he came to the General Hospital to 
see Dr. Molson. About the 10th he began to get feverish and 
extremely restless. On the 14th the temperature was above 104 , 
and he became comatose. The left arm seemed powerless ; the 
right arm and leg were constantly twitching. On the 15th the 
temperature reached 105 F., and there were cutaneous ecchymoses. 
He died on the morning of the 16th. The autopsy showed very 
extensive mitral valvulitis, the vegetations large> soft, greyish- white in 
colour. No chronic affection of the valves. The spleen and kidney 
contained many recent infarcts. The brain and membranes healthy, 
with the exception of a spot of greyish-red softening in the right 
corpus striatum (lenticular nucleus) about the size of a cherry. It 
was no doubt embolic, though the arteries of the perforated space 
were carefully examined for emboli without success. 

Case III. — -Emma M., aged 18, was admitted to the Montreal 
General Hospital, under Dr. George Ross, and died in five days of 
exhaustion. There was no rheumatism, and the attack of chorea had 
followed a fright five days before admission. Here, too, the only 
important lesion was on the mitral valves — a row of soft, warty 
vegetations on the auricular face, just within the free margins. (The 
case is given under Chorea insaniens.) 

Case IV. — Miss A., aged 27, was admitted to the Johns Hopkins 



CONDITION OF HEART IN FATAL CASES. 53 

Hospital December 24th, with violent chorea, of which she died on 
January 4th. (Case referred to fully on page 23.) The heart was 
pale and flabby, no signs of old cardiac lesion other than that the 
bases of the columnse camese of the left ventricle were somewhat 
fibroid. The edges of the mitral segments were a little thickened, 
and presented on the auricular faces a row of bead-like vegetations. 

Case V. — Female, age above 70, admitted to Johns Hopkins 
Hospital December 2nd, 1893, with chorea of three or four weeks' 
duration, which was stated to have followed mental worry and dis- 
appointment. There was a loud, blowing, systolic murmur. She 
improved a great deal, but died somewhat suddenly on the 7th of 
April, after an attack of cardiac dyspnoea. The autopsy showed old 
mitral valve disease ; no fresh vegetations. 

As is well known, the mitral segments are the most frequently 
involved. In forty-three cases they were alone the seat of endo- 
carditis ; in thirteen instances the mitral and aortic segments 
presented vegetations ; in three cases the tricuspid segments were 
involved with the mitral ; in two there were vegetations on mitral, 
tricuspid and aortic valves ; and in one case the aortic valves were 
involved alone. The tricuspid valves may be alone attacked, as in 
a case reported by Babington. 1 Acute or subacute arthritis had 
occurred in thirty- one of the cases ; in four it was doubtful, and in 
thirty- seven it was specially stated not to have been present. 

Of the fatal cases in which endocarditis was not present in two 
there was pericarditis, in two chronic mitral valvulitis, in one the 
heart was fatty. 

In the almost constant association with endocarditis fatal cases of 
chorea stand unique among diseases. The only list of fatal cases 
of rheumatic fever available with which to make a comparison is 
that of Guy's Hospital. Of forty-five cases which ended fatally, 
and in which there had been no previous chronic disease of the 
valves, the organ was found to be healthy in eight only, in nineteen 
both endocarditis and pericarditis existed, pericarditis alone in ten, 
endocarditis alone in eight. 2 The percentage of unaffected hearts 
in this series is considerably higher than in the chorea cases just 
given. Endo-pericarditis is very much more frequent in rheumatism, 
and the proportion of cases of pericarditis is very much greater. 

The endocarditis, almost invariably of the simple variety, is shown 
by the presence of a few small bead-like vegetations just within the 

1 Guy's Hospital Reports, Vol. VI, p. 436. 

2 Fagge's Practice of Medicine, Third Edition, by Pye-Smith, London, 1891. 



54 CHOREA. 

margins of the auricular surface of the mitral cusps. They present 
the usual characters of such structures, and in the cases which I 
have seen have differed in no respect from the endocarditis met with 
in rheumatism and in the secondary infections in various febrile 
disorders. I see no grounds whatever for Dickinson's suggestion 
that the bead-like vegetations in chorea are not identical with those 
in other affections. Sansom l states that there are two forms of 
endocarditis in chorea ; the ordinary rheumatic valve-thickening, 
and the beading of the cusps with papillary elevations. Thickening 
of the edges of the cusps, however, I regard as too uncertain a 
criterion, and no cases are included in the series which had not 
actual vegetations. In Case III of my autopsies, a patient of Dr. 
Molson's, of Montreal, the symptoms and lesions were those of 
malignant endocarditis. There were numerous large vegetations 
springing from the auricular edge of the segments in their entire 
extent. Those attached to the anterior curtain were the largest, and 
projected considerably beyond the margin of the valve. They were 
soft, greyish-white in colour, and irregular on the surface. Both 
spleen and kidneys presented recent infarcts. It was in this case 
that there was a small embolic lesion in the right lenticular nucleus. 
In the case reported by Friis, No. 41, and in one of Goodall's cases, 
No. 32, the endocarditis was ulcerative. 

III. — Subsequent Condition of the Heart in Choreic 
Patients. 

It seemed important to determine the subsequent heart history of 
a considerable number of cases of chorea, since it was clear that in 
this way alone could satisfactory evidence be obtained as to the 
influence exerted on it by the primary disease. Stephen Mackenzie 
has already shown in an examination of thirty-three patients at 
periods varying from one to five years subsequent to the attack 
" that indisputable heart disease persisted in 6o*6 per cent, of the 
series of chorea cases examined." Forty-four children examined by 
Donkin 2 at periods varying from two to twelve years after the chorea 
eighteen had signs of heart disease. Accordingly in the spring of 
1887, with the assistance of Dr. Charles Burr, and in the spring of 
1889, with the assistance of Dr. Caspar Sharpies, I made an attempt 
to reach all the cases of chorea which had been in attendance at the 
Philadelphia Infirmary for Diseases of the Nervous System since 

1 Lancet, January 12, 1889. 

2 Diseases of Children. London, 1893, P- 3° 2 - 



CONDITION OF HEART IN CHOREIC PATIENTS. 55 

1876. In all instances the examination was made two or more 
years subsequent to the attack of chorea. In each case reference 
was made to the original notes, questions were asked about sub- 
sequent attacks and rheumatism, the heart was examined in the 
recumbent and erect postures, at rest and after exertion, and the 
notes were dictated at the time of the examination. The results of 
the examination of the first series, comprising no cases, have 
already been published, 1 and sufficient details given of the cases to 
indicate the nature of the heart lesion. 

Of the 140 cases 98 were in females, 42 in males. The length 
of time which had elapsed since the attack varied in the cases 
examined from sixteen years to two years. In the first series more 
than half (63) of the cases were examined at a period of five or 
more years subsequent to the attack. The results of the examina- 
tion were as follows : — In 5 1 cases the heart was normal ; in 1 7 
there was disturbance which might reasonably be regarded as 
functional ; in 72 cases there were signs of organic heart lesion. 
This gives a remarkable, and I must say at the time, an unexpected 
high percentage (5 if per cent.) of cases in which following an 
attack of chorea there was definite damage apparent in the heart. 

Normal Cases. — Of the 51 persons in whom the heart was 
found to be normal, 15 had three or more, 8 had had two attacks, 
and 27 a single attack. There was a history of rheumatism in 9 of 
the cases. In 7 of these the attack was of the acute articular type. 

Functional Cases. — Of the cases presenting abnormal signs 
17 may reasonably be cited as examples of functional dis- 
turbance. They were cases without enlargement of the heart 
and with localized or variable murmurs. Eleven presented soft 
apex systolic bruits, not propagated to the axilla, and in four 
varying with the position of the patient. In most of these cases 
there was also accentuation of the second sound in the second left 
interspace, a feature upon which, however, no special stress can be 
laid in young persons, since in them it is by no means uncommon 
in perfectly normal hearts. Comparison between the intensity of 
the sounds in the second right and second left interspaces was a 
point upon which particular attention was paid in examination of 
the cases. Thus in ten of the normal cases the pulmonary second 
was distinctly louder than the aortic, and in several instances 
reduplicated. No stress was laid upon the cardio-respiratory 
murmurs, which are common in thin-chested young children. 
1 American Journal of the Medical Sciences, 1887, L 



56 CHOREA. 

In two of these cases with functional disturbance the sounds in the 
pulmonary area were clear in the erect posture, but when the patient 
was lying down a systolic murmur was present ; in both the second 
sound was accentuated, and in one the area of pulsation somewhat 
increased. In a third case there was a soft, systolic murmur in the 
second and third spaces in the recumbent position, one with 
accentuation of the second sound and an apex beat a little outside 
the nipple line. There may have been in some of these instances 
organic changes in the valves, but it was deemed best to exclude all 
those in which the signs were doubtful. 

Cases with Signs of Organic Disease. — Of the seventy-two 
cases thirty had had three or more attacks of chorea. The question 
of rheumatism was carefully investigated in each instance. In 
twenty-five of the cases, 34*13 per cent, there was a history of acute 
arthritis, which in seven of the cases had followed the chorea in 
from one to five years. Comparing the frequency of the articular 
affection in this group, 34*13 per cent., with that in the total series ; 
namely 24*2 per cent., or with the group of fifty-one normal cases, 
in which it was 17*11 per cent., we see illustrated the greater 
liability to serious heart mischief in the cases with joint com- 
plications. We have, however, the much larger proportion, 66 per 
cent, of cases with positive organic disease examined at a date two 
or more years subsequent to the attack of chorea, and questioned 
carefully as to the occurrence of rheumatism, in whom there was no 
history of the existence of this complication. 

Nature and Seat of tlu Lesion. — In a large proportion of all 
the cases the signs were those of mitral valve disease, usually in- 
sufficiency, indicated by the systolic murmur of maximum intensity 
at the apex, propagated to the left, with evidences of enlargement of 
the heart, sometimes also by a thrill, and marked accentuation of 
the second sound in the pulmonary region. The details of a large 
series of these cases are presented in the paper above referred to. 
While in a majority of the cases there given the lesion was well 
compensated, there had been in not a few instances attacks of 
shortness of breath and palpitation. Three of the cases died with 
the symptoms of chronic valve disease. In twenty-four instances a 
mitral pre-systolic murmur was present. In one of these instances 
(Case LXXXII in the already published series) it was possibly the 
apex diastolic murmur heard in connection with aortic insufficiency, 
the so-called Flint murmur. The young man, aged 18, had his first 
attack of chorea in 1884, a second attack in 1886, and in 1887 a slight 



CASES WITH SIGNS OF ORGANIC DISEASE. S7 

attack of rheumatism. There was a soft murmur at the base in his first 
attack. In 1887, when examined, there was a loud diastolic murmur of 
maximum intensity on the sternum, and heard also at the aortic carti- 
lage. At the apex there was a rumbling pre-systolic murmur, localized, 
and not accompanied with a thrill. One of the cases with mitral 
presystolic murmur subsequently died. The lad, aged 13 (Case 
XXXIX of the series published) had his first attack of chorea in 
1881, when seven years old. He had a second attack in 1882 and 
a third in 1884. He never had any joint troubles. Of this, both 
his father and mother, who were exceedingly intelligent people, were 
positive. A half-brother has, however, had rheumatic pains in the 
joints. He had scarlet fever when three years old, but not severely. 
With this exception he had had no other diseases except chorea. 
He had, when examined, great shortness of breath and more or less 
lividity with a distinct pre-systolic thrill in the fourth interspace, a 
blubbering pre-systolic murmur, also of maximum intensity, in the 
fourth space, and a loud, blowing systolic bruit transmitted to the 
axilla. There were signs of considerable hypertrophy of the heart. 
The autopsy showed extreme stenosis of the mitral orifice with great 
dilatation of the left auricle and enormous hypertrophy of the right 
ventricle, with beginning contraction of the tricuspid orifice. 

The importance of chorea as a factor in the etiology of chronic 
mitral valve disease is shown in a striking manner by the examina- 
tion of these cases. It is unfortunate that it is not always inquired 
for in the anamnesis of heart cases in the medical wards. It is 
interesting to note the comparative rarity of the involvement of the 
aortic valves. There were only four instances of combined aortic 
and mitral valve disease. This is in accord with the anatomical 
distribution of the endocarditis, which has been shown to be so 
much more frequent on the mitral segments. 

It may be then stated : 

(1) That endocarditis is a very common complication of chorea 
minor. 

(2) That in a majority of such cases the endocarditis is 
independent of, and is not associated with, acute arthritis, unless 
indeed we regard the valvular lesion as itself a manifestation of the 
rheumatism, holding with Bouillaud that " chez les jeunes sujets le 
cceur se comporte comme une articulation." 

(3) That in a considerable proportion of cases, much larger 
indeed than has hitherto been supposed, the complicating endo- 
carditis lays the foundation of organic heart disease. 



58 CHOREA. 

IV. — Pericarditis in Chorea. 

Inflammation of the pericardium in connection with chorea was 
first described by Bright, who states also that this had been long 
observed by Guy's Hospital physicians. His statement of the 
matter is worth quoting : " With regard to the connexion between 
chorea and inflammation of the pericardium, when called upon the 
year before last to deliver the Lumleian Lectures at the College of 
Physicians, I took occasion to state, that for some years I had been 
persuaded of the existence of such a combination, and little atten- 
tion has hitherto, as far as I know, been paid to the subject, 
although the combination of this spasmodic disease with rheumatism 
has been long recognized. In the very excellent " Syllabus, or 
Outlines of Lectures on the Practice of Medicine," published at 
Guy's Hospital, I find, in the edition of 1802, rheumatism distinctly 
stated as one of the existing causes of chorea ; and in later editions, 
as in that of 1820, I find it stated, that "chorea sometimes 
alternates with acute rheumatism," but through what organ or by 
what intervention this occurs is not conjectured. 1 He gives in this 
paper five cases of pericarditis with chorea and rheumatism ; in the 
first case there was also endocarditis. The Lumleian Lectures for 
1836, to which he refers, are not in the Lancet of that year, nor can 
I find that they were published. Bright thought the connection 
between the two was through the phrenic nerve, which communicated 
the irritation from the inflamed pericardium to the spinal cord. By 
far the best account in the literature is that by Sibson, 2 in his 
exhaustive article on pericarditis. He states that 21 of the 180 
cases of acute rheumatism with affections of the nervous system had 
chorea. Fifteen of those patients with chorea had pericarditis, six 
had no pericarditis ; while fourteen of them had endocarditis ; three 
had no endocarditis, and in three of them endocarditis was pro- 
bable or doubtful. In 19 of the 73 recent autopsies in chorea 
which I have collected, pericarditis occurred as a complication, and 
in seventeen it was associated with endocarditis. In eight of the 
cases there was a history of acute rheumatism (arthritis). 

One case had sub-acute rheumatism, one rheumatic pains, while 
nine had not had acute arthritis. 

Of the nineteen cases eight were under ten years of age, eleven 
were in the second decade. 

1 Med. Chir. Soc. Trans., Vol. XXII. p. 10. 

2 Reynolds' System of Medicine. 



PERICARDITIS IN CHOREA. 59 

In three the pericarditis was old ; in three death is stated to have 
been caused directly by the pericarditis ; pleurisy occurred in five 
cases, pneumonia in four ; acute phlebitis in one, and in one acute 
nephritis. 

The following is a very typical case from the records of the 
Infirmary. I saw it repeatedly with my colleague, Dr. Sinkler, 1 who 
has reported it. 

George W., aged 6, applied at the Infirmary October 29th, 1888, 
with chorea. Family history was good. He had been well up to 
February 1888, when, after exposure to cold, he had an attack of 
inflammatory rheumatism. In March the choreic movements began, 
and had persisted ; when he came under observation they involved 
the face, trunk, arms and legs, and the speech was somewhat 
disturbed. 

On November 28th he was admitted to the wards, and under rest 
and Fowler's solution the movements became less marked. 

On January 6th, 1889, he had a severe pain in the umbilical 
region, and on the 7 th in the right side. This persisted throughout 
the 8th and 9th. On the 9th I made the following note : — " Res- 
piration 18; pulse 96, volume small, increased tension. The 
praecordial region is rather prominent ; the heart's impulse is felt in 
the third and fourth interspaces, the maximum impulse in line of 
nipple ; no thrill is felt. The vessels of the neck are prominent, 
full and pulsating ; praecordial dulness begins above at the third rib ; 
externally, a little outside of nipple line ; to the right it extends two 
fingers'-breadth beyond the sternum. On auscultation, a loud 
systolic murmur is heard at the apex and is transmitted to the 
scapula. Over the whole prsecordial space a loud to-and-fro murmur 
is heard. The sound is intensified at the base in the third and 
fourth interspaces ; it is well heard at the nipple and at the ensiform 
cartilage." Following this the patient had a well-marked effusion, 
which gradually disappeared, but he was a long time convalescing, 
and the chorea did not disappear entirely until June. During this 
time he had no arthritic manifestations, and no subcutaneous fibroid 
nodules. The temperature throughout the attack was high and 
sometimes reaching 104 . 

1 University Medical Magazine, Vol. II, p. 483. 



6o CHOREA. 



CHAPTER V. 

MORBID ANATOMY, PATHOLOGY, AND TREATMENT. 

Statistics of Fatal Cases — Changes in the Nervous System — Bacteriology — 
Pathology — Treatment. 

MORBID ANATOMY. 

There are no characteristic lesions in fatal cases of chorea. 
Externally there, are frequently bruises and excoriations. In cases 
of long duration the body is much emaciated, while in acute 
cases with high fever the appearances are those of a person dead of 
an infectious disease — the skin is congested, the blood is dark, and 
the muscles are of a very red colour. 

The statistics of fatal cases collected by Sturges and by Raymond 
have been referred to in the section on the heart in chorea. Of the 
73 cases which I have collected since the publication of their figures 
the general analysis is as follows : — 23 were in males, 50 in females ; 
15 occurred in children of 10 years and under; between the ages of 
11 and 15 inclusive there were 17; between 16 and 20 inclusive 
there were 313 between 21 and 30, there were 6; three cases 
occurred in the aged, and in one the age was not given. More than 
double the number occurred in the fourth hemi-decade than in the 
first two hemi-decades. The ratio of non-fatal cases in these periods 
is 1 to 4*2. 

There was a history of acute arthritis in 29, in three there had 
been sub-acute rheumatism, and in four the history was defective. 
Four cases were in pregnant women, and one occurred post-partum. 
As already stated in the section on the heart, recent endocarditis 
was present in 62 cases, 85 per cent. ; and pericarditis in 19 ; in two 
pericarditis alone ; in two chronic mitral endocarditis ; and in one 
the heart was fatty. There were in all 66 cases with heart lesions, 
90*4 per cent. 

Associated Lesions. — Pneumonia occurred in nine cases usually 
lobar in type, but in several cases lobular ; acute pleurisy was noted in 
seven cases ; peritonitis in one case ; parotitis in two ; phlebitis 



MORBID ANATOMY. 6 1 

in two; purulent bronchitis in No. 41; pyaemia (or septicaemia) 
occurred in Nos. 4, 47, and 57. 

Among the incidental lesions may be mentioned hsematoma of 
the dura mater (Nos. 16 and 51) ; acute nephritis (Nos. 31 and 60) ; 
gangrene of the foot (No. 32). One patient (No. 25) is said to have 
died of acute dilatation of the stomach. 

It is not easy to say accurately just how many died of the disease 
itself and how many of the complications. 

NerYOUS System. — Naturally special attention has been paid to 
the condition of the brain and spinal cord. Dana 1 has carefully 
analyzed the recorded autopsies, of which in only 39 was the con- 
dition of the nervous system at all satisfactorily described. Of 19 
cases in which careful microscopical examinations were made, in 
16 there was intense cerebral hypersemia, peri-arterial exudations, 
erosions, softened spots, minute haemorrhages, and occasional 
emboli. The changes were most marked in the deeper parts of the 
motor tract, particularly the lenticular nuclei and the thalami. These 
vascular changes, perhaps the most constant central lesions of 
chorea, are essentially the same as those described by W. H. 
Dickinson in 1876. In Autopsy IV of my series they are very fully 
described by Berkeley. 2 The peri-vascular spaces were wide, filled 
with round cells, occasionally imbedded in hyaline masses. The 
largest transudations were about veins. The adventitia was often 
covered by small round cells and hsematoidin debris. The muscu- 
laris was not hypertrophied. The intima was thickened in spots 
and presented small swollen nuclei with numerous refractile granules 
in their substance. 

In No. II of the fatal cases of my series, in which the mitral 
endocarditis was very extensive, there was a spot of embolic soften- 
ing the size of a cherry in the right lenticular nucleus. It was upon 
the presence of lesions of this kind in connection with endocarditis 
that the embolic theory of the disease was suggested and supported 
by Kirkes, and after him by Broadbent, Tuckwell, and others. 

There are two cases reported in which death took place from 
apoplexy, one by Bevan Lewis, 3 in which there was cerebellar 
haemorrhage ; in the other by Baxter, 4 a girl of eight years, the 
haemorrhage was extraventricular. 

Of special histological features the following may be mentioned : — ■ 

1 Brain, Vol. XIII, 1890. 

2 The Johns Hopkins Hospital Reports, Vol. II, p. 325. 

3 Medical Times and Gazette, 1876, II. K Brain. April, 1879. 



62 CHOREA. 

(a) Chorea corpuscles (so-called). These bodies were described 
by Elischer 1 as sharply contoured, irregular, strongly refractile, con- 
centrically laminated bodies, attached to the blood-vesssels in the 
corpora striata and internal capsule. Jakowenko 2 described them 
in six cases from Flechzig's clinic. Flechzig himself seemed to lay 
considerable stress upon their significance. 

Wollenberg 3 examined carefully six brains from cases of chorea 
minor with particular reference to the presence of these bodies, and 
in addition forty-six brains of non-choreic cases. He concludes that, 
in some cases of chorea, these highly refractile bodies occur in the 
neighbourhood of the lenticular nucleus, arranged along the vessels, 
but that they are in no way characteristic, as they also exist in 
similar situations in individuals who have never suffered with chorea. 
He thinks that these bodies represent some calcified organic sub- 
stance of unknown nature. 

{b) Lesions of the pyramidal cells. F. Charlewood Turner 4 has 
described in the brains of five cases swelling and turbidity of certain 
of the larger pyramidal cells in the deeper layer of the cortex in the 
Rolandic region. "The protoplasm is cloudy and dense-looking, 
and more fully stained than in normal cells, and defined at the bases 
of the processes, which would indicate a great swelling up of the body 
of the cell." 

Berkeley, in the case above mentioned, could find no special 
changes in the nerve cells. The peri-cellular spaces were large, but 
the chief changes found were meningo-vascular, and endo-arterial. 

Dana, 5 in a recent case, which, however, was associated with 
chronic lepto-meningitis, found hyaline degeneration of the pyramidal 
cells. 

(c) Lesions of the medulla and spinal cord. No characteristic 
changes have been met with in these parts. Berkeley found many 
foci of minute haemorrhage in the pons, especially among the fibres 
of the pyramidal tract. The blood-vessels showed the same changes 
as in the cortex. "The nuclei on the floor of the ventricle, and 
more anteriorly the olivary bodies, the internal fibres of the vagus 
and hypoglossus, as well as the transverse fibres, were examined with 
great care and patience, but without finding the slightest pathological 

1 Virchow's Archiv, Bd. LXIII. 

2 Reference in Neurologisches Centralblatt, Bd. VIII. 

3 Archiv filr Psychiatrie und Nervenkrankheiten, Bd. XXIII, 1891, p. 167* 

4 Pathological Society 's Transactions, Vol. XLIII, 1892. 

5 American Journal of the Medical Sciences, 1894, I- 



NERVOUS SYSTEM. 63 

change in them." In the spinal cord the multipolar cells were 
mainly perfect in protoplasm, nucleus and prolongation. In canine 
chorea, an affection entirely distinct, however, from chorea minor 
in man, H. C. Wood has described changes in the ganglion cells. 
" When the animal was killed in the very beginning of the attack, 
the cells showed no change ; a little later the only alterations in the 
cells were the very frequent absence of the nuclei, the failure of granula- 
tions in the protoplasm, the loss of power to take staining fluids, and 
rarely the occurrence of sharply-defined vacuoles. Then the pro- 
cesses began to drop off; and finally it was found that the places of 
the cells were occupied by irregular, globose, crumpled-looking 
masses, without sharp outline, and taking carmine staining very 
faintly. No granulations, no nuclei, no processes, were apparent. 
These masses represent the cells in the last stages of degenera- 
tion." 1 

Berkeley, 2 in a dog which had had chorea for between five and six 
months, found the cells of the spinal cord in all respects perfectly 
normal. 

Triboulet, 3 however, who has made a very careful examination of 
the spinal cord in cases of canine chorea, states that he has con- 
firmed the observations of Wood, and finds very marked lesions in 
the cells of the anterior horns. He agrees, however, that the canine 
chorea is an entirely different disease from that seen in man. 

Bacteriology. — Attempts have been made to isolate micro- 
organisms in chorea. From what we know of the invasion of the 
body by bacteria, we should expect to obtain cultures in the acute 
febrile cases. Such a series of observations as that of my colleague 
Prof. Welch 4 on the organisms found in cultures made from 180 
autopsies shows how cautious one should be in judging of the 
etiological value of the presence of certain forms. 

The observations which have been made so far on chorea are 
not at all satisfactory. Naunyn found a cladothrix on the meninges 
and in the endocardial vegetations. 

In the fatal case from my wards reported by Berkeley, the cultures 
made from the blood in the left ventricle, from the vegetations on 
the valve, and from the parotid gland, showed an abundant growth 
of the staphylococcus pyogenes aureus. 

1 Nervous Diseases and their Diagnosis, Philadelphia, 1887, p. 155 ; also 
Therapeutic Gazette, 1885. 

2 Johns Hopkins Hospital Reports, Vol. II, p. 33J. 

3 Paris Thesis, 1893. * Middleton- Goldsmith Lecture, 1894. 



64 CHOREA. 

Triboulet 1 deals extensively with the possible infective character 
of chorea, and has made a number of cultures, in two fatal cases 
finding the staphylococci; in another instance he found staphy- 
lococci in the blood during the febrile attack. 

The most exhaustive research on the subject is that by Pianese" 
from the Pathological Institute of the University of Naples. He 
claims to have isolated a bacillus from the nervous system of a choreic 
patient, which he was able to cultivate successfully. Animals 
inoculated died with muscular twitching and convulsions, and from 
these animals the same bacillus could be obtained in pure cultures 
from the central nervous system. His work, a copy of which he 
very kindly sent me, is illustrated by beautiful plates showing the 
cultures and the micro-organisms in the tissues. 

Dana :> has recently reported a case, not, I should suppose, of 
Sydenham's chorea, in which in the meninges a diplococcus was found 
resembling the diplococcus lanceolatus. A few other observations 
are found in the literature, as Richter, 4 who found cocci in the 
blood, and Donkin, 5 who found rod-like bodies in the tissues. 
Altogether the only observations which lay claim to anything like 
completeness are those of Pianese, and they await confirmation. 

PATHOLOGY. 

A satisfactory presentation of the pathology of chorea cannot be 
given in the present state of our knowledge ; and now, at the close 
of the century, as at the beginning, in the time of Bouteille and 
Bemt, there are many problems awaiting solution. While our 
clinical information has widened and has become more accurate, 
the quotations from these old worthies, which I give on page vi, 
express the judgment of our own day on the obscure nature of the 
malady. 

It is not my intention to discuss the various views which have 
been broached ; the student and the specialist will find them fully 
considered in the larger works on diseases of the nervous system, 
particularly in that of Gowers, and in the recent monograph of 
Sturges (2nd edition). I have thought it best to take up the recent 
suggestion that chorea minor is an infectious disorder, and to present 
some of the points which are urged in favour of this view. 

1 Paris Thesis, 1893. 

2 La Natura Infettiva della Corea del Sydenham. Ricerche anatomiche speri- 
mentali e clinic he del Dottore Giuseppe Pianese. Naples, 1893. 

3 American Journal of the Medical Sciences, January, 1894. 

* Western Lancet, Vol. XII. * Medical Times and Gazette, 1884. 



PATHOLOGY. 65 

The severer types of Sydenham's chorea have all, the milder 
forms some, of the features of an acute infectious disease, and were 
these graver types alone seen, no one would question its position ; 
but in the slighter varieties the suggestion seems strained, while the 
psychical element in causation, often so striking, is very strongly 
opposed to any such theory. The role of infection in producing 
diseases of the nervous system is recognized as of growing importance. 
The infections may be thus grouped : — 

(a) Tuberculosis and cerebro-spinal meningitis, in which the in- 
fective organisms are present in the lesions. 

(b) Diphtheritic paralysis, tetanus, and possibly hydrophobia, in 
which the toxic materials are alone present. 

(c) Syphilis, of which neither the organism nor its toxine is 
known, and which has the remarkable dual action, the one proxi- 
mate, inducing gummata and arteritis ; the other remote, causing 
scleroses. 

(d) Then comes a group of diseases in which infection is 
possible, but for which satisfactory evidence is still lacking : — some 
forms of acute neuritis, acute polio-myelitis, acute encephalitis, and 
here some authors would place chorea minor. 

The following points favour the view that chorea is an acute in- 
fection : — 

1. The influence of age, sex and season, in which it presents the 
peculiarities common to infectious disorders. 

2. The clinical course of the severer forms, in which not a single 
feature is wanting of a typical infection. The disease presents, as 
occurs in infections, all gradations between the mild form with 
scarcely an unpleasant symptom, to cases of such intensity, as in the 
one reported by Cook and Beale, that death may occur in 130 hours. 

3. The post-mortem appearances. Endocarditis, the most con- 
stant change, upon the significance of which not sufficient stress has 
been laid by writers, is one of the most distinctive lesions of an in- 
fectious disease. Varying in frequency in different disorders, and 
produced by a variety of organisms, still, so far as we know, it does 
not occur apart from the conditions resulting from what we designate 
as an " infection." In the opinion of many good observers its presence 
is really enough to stamp the nature of chorea. Other lesions, such 
as pericarditis, pleurisy, parotitis, septic inflammation, point in the 
same direction. 

Intimately associated with this question is that of the relation of 
chorea to acute rheumatism. Are its symptoms merely manifesta- 

F 



66 CHOREA. 

tions of the rheumatic poison, or does the arthritis bear the same 
relation to chorea as the joint inflammation to gonorrhoea, or to 
cerebro-spinal fever ? 

One of the " vulgar errors " of the profession has been the abuse 
of the term rheumatism, which, almost in the same way as chorea, 
has been used to cover a multitude of totally different affections. 
Acute rheumatic fever, an acute infection of unknown origin, is a 
well-characterized and only too common disease ; but all cases of 
acute poly-arthritis are not rheumatic fever. There are many in- 
fectious diseases, in which arthritis develops as a secondary com- 
plication — scarlet fever, typhoid fever, cerebro-spinal fever, dysentery, 
septicaemia and gonorrhoea. 

Gonorrhoea is an acute local infection, often accompanied by 
arthritis, and sometimes complicated with intense pericardial and 
endocardial inflammation. No one now believes that this arthritis 
is identical etiologically with acute rheumatism. The pain, the 
redness, and the swelling may be the same, but the general 
behaviour, the irregular localization, the greater liability to suppura- 
tion, the protracted course, the resistance to the salicine compounds, 
all point to a different cause. 

There is an acute affection of the nervous system directly com- 
parable with chorea in this very matter of acute arthritis. In 
epidemic cerebro-spinal meningitis, arthritis occurs in a variable 
percentage in different outbreaks ; thus, in the recent one reported 
upon by Flexner and Barker, 1 " nearly twenty per cent, of the severe 
cases suffered from complicating joint affections, the knees being 
most frequently affected, the elbows, wrists, ankles following in 
frequency in the order named. The effusions were peri-articular as 
well as articular, and the joints were swollen and red, resembling 
closely the appearance of those in acute rheumatic arthritis ; indeed 
there were cases which, had it not been for certain initial symptoms 
indicating the meningeal process, could have easily been diagnosed 
as nothing more than attacks of rheumatism." They note also that 
the cerebral symptoms appear to be favourably influenced by the 
joint affections. There is scarcely an argument used by the strong 
advocates of the rheumatic nature of chorea which could not be 
applied most forcibly in favour of the rheumatic origin of epidemic 
cerebro-spinal meningitis. Some indeed would come with telling 
force, such as the almost invariable association of the disease with 
inclement weather and changeable seasons. 

1 American Journal of the Medical Sciences, 1894, I. 



PATHOLOGY. 6j 

The nature of the virus of rheumatic fever is unknown. Several 
forms of micro-organisms have been found associated with acute 
poly-arthritis, and in one instance, with all the features of an acute 
rheumatic fever, the micrococcus lanceolatus was present. A careful 
study of the bacteriology of the disease has recently been made by 
Professor Sahli, 1 of Bern. He regards the acute onset, the self- 
limited course, the complication and the frequency of arthritis in 
other infections, the similarity in many respects of the disease to a 
septicaemia — all as supporting the view that it belongs to this group. 
He has found in some cases an organism identical with the 
staphylococcus citreus, but of very low virulence. In connection 
with this point Flexner and Barker make an interesting suggestion 
in discussing the joint lesions of cerebro-spinal meningitis. 
" Nevertheless it is probable that acute articular rheumatism will, ere 
long, be proven to have no etiological u?iity, just as has already been 
proven for the inflammations of the serous membranes generally. 
It seems probable that the entrance of pyogenic organisms of 
different varieties into the circulation, under circumstances which 
are inconsistent with the development of the phenomena of a general 
septicaemia, may give rise to inflammations in some one or more 
of the serous membranes of the body — be it meninges, pleurae, 
pericardium, peritoneum, or joint surface, the particular ones attacked 
depending on certain peculiarities either in the virulence of the 
invading organism or in the lessened resistance at the moment of the 
serous membrane implicated." 

Evidently we are as yet upon the threshold only of our know- 
ledge of the essential cause of either acute rheumatism or chorea. 
In both disorders there are facts highly suggestive of an infective 
nature, but more than this cannot be said at present. The relationship 
of the two diseases remains unsolved. If, as some would have it, 
chorea is only one of the rheumatic states, we have to stretch beyond 
recognition our conception of the disease, now, in the absence of a 
knowledge of its etiology, necessarily characterized by its symptoms. 
Very probably the cause of chorea will be found to be a poison 
allied to, but not the same as, that of rheumatism. On this question 
of the infective nature of the disease Gowers very justly remarks 
that when the causation of a disease is complex it often happens 
that at different periods attention is directed too exclusively to one 
of the elements of its production. 

While, on the one hand, the course of the milder forms, and the 
1 Dentsches Archil) fur klinische Medicin, Bd. LI. 

F -2 



68 CHOREA. 

not infrequent association with fright, favour the view that the 
disease is a simple neurosis ; on the other, the course of the severer 
forms, the arthritis, and the almost constant presence of endocarditis 
in the fatal cases, speak for an acute infection. 

It is interesting to note that the evidence in favour of the 
infectious character of canine chorea is now very strong. 1 

The site of the primary change in chorea minor has been much 
discussed. That the condition found in fatal cases gives no satis- 
factory information on this point is evident, since, basing their 
arguments on these findings, authors have claimed in turn the cortex, 
the basal ganglia, and the cord as the seat of the disease. As in 
the consideration of the nature of the disease, the aggravated cases 
give us such important information, so with respect to the seat of the 
primary change, the intense psychical phenomena indicate clearly 
a cortical lesion. The alterations in character, and the mental 
features of the malady in less severe forms, also suggest involvement 
of the higher centres. The cessation of the movements during 
sleep, and the frequent occurrence of hemi-chorea, are usually urged 
as favouring this view. Of course, irregular movements of muscles 
may be produced by irritation in any part of the motor path, but 
" the motor impulses that excite the muscles pass to the spinal cord 
from the motor region of the cortex. It is here that movements 
are arranged, and if they are disarranged and the disorder proceeds 
from the brain, we naturally refer it to a disordered action of th e 
cells of the cortex " (Gowers). The part played by the spinal 
cord is probably subsidiary in the chorea of man. H. C. Wood 
urges that the ganglion cells in the whole cerebro-spinal system suffer, 
and he has adduced experimental evidence to prove that in the dog 
at least the cord is chiefly involved. But, as already remarked, 
canine chorea is a different disease from chorea minor in man. 
From time to time cases are reported which suggest the spinal cord 
as the seat of the lesion, but, as in those recorded by S. Weir 
Mitchell and Burr, 2 they are usually not cases of chorea minor. 

TREATMENT. 

Of the conditions favouring the development of the disease three 
are important and may be guarded against : — 

(a) Brain and eye-strain at school. These are perhaps the most 
important, and a child who has had one attack of chorea minor 

1 Triboulet, Paris Thesis, 1893. 

2 Journal of Nervous and Mental Diseases, 1890, XV. 



TREATMENT. 69 

should be prohibited from competing for prizes in class work, and 
should have the lessons carefully regulated. 

(b) In children of families subject to rheumatism very special care 
should be taken, particularly in the spring months, that there is no 
unnecessary exposure to cold and damp. 

(c) The nutrition of the child should be maintained at a 
maximum, and on the first appearance of anaemia, iron and arsenic 
should be used. These are measures which can be urged upon a 
mother whose child has already had an attack. 

The most important elements in the treatment of the attack itself 
are rest and seclusion. The child should be put to bed and kept 
there until the movements have ceased. This may seem an un- 
necessarily severe procedure for a disorder so simple, apparently, as 
chorea minor, but the very mildest cases are not without danger, and 
it is probable that the liability to heart complication is considerably 
diminished by complete rest. As the movements diminish the child 
may be moved to a lounge. The remarkable influence of this 
procedure in allaying the severity of movements is often seen in 
hospital practice, and a case which in the out-patient department 
has seemed of extreme severity, has, at the end of two or three days, 
been, without any medication, changed to one of comparative light- 
ness. In private practice it is a measure usually resented by mothers, 
and may be very difficult to carry out. I have often insisted, where 
the family could afford it, upon the presence of a special nurse. 

Next to rest, perhaps the most valuable measure is seclusion. 
Usually the children affected are very bright and intelligent, and 
correspondingly sensitive, and the consciousness of their affliction is 
a constant source of worry and irritation. The child should not 
see many persons, and when possible should be in charge of either 
the mother or an intelligent nurse, whose main occupation should 
be to read to the child and keep it amused. 

In the severer cases with great jactitation it becomes a question of 
protecting the child from injury. The mattress should be very soft, 
and all neighbouring hard objects should be guarded with soft 
cushions and pillows. It is sometimes well to cover the elbows, 
knees and ankles with cotton-wool. In the terrible cases of chorea 
insaniens the liability to bed-sores is very great, and a water-bed may 
be necessary. It is well to remember that in children the move- 
ments may be such that the child is thrown out of bed, an accident 
which I have known to occur several times in hospitals, in which 
the beds are as a rule very unsuitable for choreic patients. It is 



JO CHOREA. 

sometimes better in a severe case to make up the bed on the 
floor in a corner of the room. 

The diet should be abundant and nourishing. Sometimes the 
question of feeding may become a very difficult one, when the head 
is in a constant state of movement. An ordinary baby's bottle with 
a nipple and a piece of rubber tubing may then be necessary. Milk, 
broths, nourishing soups, and eggs can be given in this way. Except 
in the very light cases, it is best to have the child fed by a nurse. 

Systematic massage and various movements may be employed 
when possible. In aggravated cases it is out of the question, but 
when the movements are not very violent thorough inunction with 
oil or cocoa-butter may be practised daily for half an hour. The 
full warm bath is often very grateful, and I have seen the movements 
diminish in the bath and after its use. Of course great care must be 
taken to prevent the child knocking itself when in the tub. In the 
severer cases of chorea insaniens the cold bath or the cold pack may 
be employed, particularly if there is high fever. 

There are no remedies which directly control the course of the 
disease, and which can be called curative. Among remedies which 
modify the symptoms and appear to do good the following are the 
most important. 

Arsenic. — For years this remedy (recommended by Thomas 
Martin, 1 of Reigate) has been employed in chorea, and it is probably 
the safest to give as a matter of routine practice. It is well to begin 
with small doses, three minims, after meals, of the Fowler's solution 
well diluted. This may be increased two minims every third or 
fifth day until the child is taking twelve or fifteen minims three times 
a day. Martin says that he " began with five drops and increased 
one drop every day until it might begin to disagree with the 
stomach or bowels," which was usually when a dose of fourteen 
drops was reached. The dose was then diminished and continued 
at ten drops for six weeks. The general condition of the children 
is often much improved by this drug, but it is extremely difficult to 
say whether it controls in any way the choreic movements. I do 
not myself think that it has very much influence upon them. When 
large doses are given the effects must be carefully watched, and the 
arsenic should be stopped on the first indication of any toxic 
symptoms, such as vomiting, diarrhoea, itching of the eye-lids, oedema, 
or skin eruptions. Pigmentation of the skin is occasionally met 
with ; a more serious effect is neuritis, of which there have been 
1 Medico-Chiritrgual Transactions, Vol. IV, 1813. 



TREATMENT. J I 

recently several cases reported. Considering the rarity of this toxic 
action upon the nerves, the cases in which it occurs must be regarded 
as instances of idiosyncrasy. Thus Marshall reports 1 a case of neuritis 
and pigmentation in a boy of six years of age, who had taken Fowler's 
solution of arsenic, ttl x t. d., for about five weeks. 

The zinc compounds were much used during the first half of this 
century. 

Of other remedies cimicifuga, antipyrin in full doses, that is from 
twenty to sixty grains in the day, chloral, exalgine, sulphonal, and 
physostigmine have all been warmly recommended. Recently H. C. 
Wood has urged the use of quinine in large doses, and has reported 
very favourable results. 

Certain features and complications require special treatment. In 
the cases with arthritis the joints should be wrapped carefully in cotton- 
wool, and opiates may be needed to allay the pain. The salicylates 
may, of course, be tried, but in the arthritis associated with chorea 
we rarely see the prompt and satisfactory results so constant in acute 
rheumatism. Should pericarditis or endocarditis supervene, the ice- 
bag may be kept applied to the prascordia. It allays the cardiac 
excitement, and in the former condition checks, I believe, the 
tendency to exudation. 

In the chorea insaniens hydrotherapy should be thoroughly tried, 
either in the form of the wet-pack or the bath. In order to apply 
the pack or put the patient in the bath it may be necessary at first 
to give chloroform, which in any case may be used with safety when 
the jactitation is excessive. 

Good results have been reported by Bastian and by Gairdner in 
these cases by large and increasing doses of chloral hydrate, keeping 
the patient continuously under its influence. The bromide of potassium 
may be combined with it. In these cases, too, the heart becomes 
feeble, and there is great prostration, for which alcohol should be 
given freely. Gee, 2 who has reported six recoveries in seven cases, 
thus sums up his experience of chorea insaniens : "to prevent bed- 
sores, to keep the patients clean and to feed them are the most 
important parts of the treatment." 

In the chorea occurring during pregnancy the same general 
treatment should be carried out, particularly the quiet and seclusion. 
When the jactitation is extreme the patient may be kept under the 
influence of chloroform. The induction of premature labour is 
recommended in very severe cases. 

1 Lancet, 1890, I. 2 St. Bartholomew's Hospital Reports, Vol. XXII. 



72 CHOREA. 



CHAPTER VI. 

CHOREIFORM AFFECTIONS. 

Habit Spasms, Tic. 

I. Simple Tic — Habit Spasm or Habit Chorea — Generalized Tic. II. Tic with 
Imperative Ideas, &c. III. Complex, co-ordinated forms of Tic. IV. Cases 
of Noisy Spasm of Respiratory Muscles. 

I propose in this section to give a brief description of those 
forms of spasmodic contraction of the muscles which are known as 
habit spasms, habit chorea and tic. It is best, perhaps, to employ 
the latter term, extending the Anglo-American usage so as to 
embrace not alone the local spasms of the facial muscles in children, 
but the more extended co-ordinated movements described by the 
French as tics convulsifs and tics co-ordonnees. Their consideration 
here is appropriate on account of the frequency with which the cases 
are confounded with chorea minor. 1 

Litre's definition of tic, "A local and habitual convulsive move- 
ment, a contraction of certain muscles, particularly those of the 
face," has been much extended by the Salpetriere school ; and 
under this term is now embraced a series of disorders of mus- 
culation, simple or co-ordinated, and with or without psychical 
manifestations. Guinon, in his elaborate article in the Dictionnaire 
Encyclopedique, gives the following definition : — " An habitual and 
conscious convulsive movement, resulting in the contraction of one 
or more of the muscles of the body, reproducing, most frequently in 
an abrupt manner, some reflex or automatic action of common 
life." The action may be controlled, or at least modified, to some 
extent by an effort of the will ; and in this, as in the reproduction 
of a movement, reflex or automatic in character, it differs from the 

1 Charcot very aptly says {Lecous du Mardi, 1888-89, P- 464) : — " Entre le tic 
.et la Choree il y a un abime : ne l'oubliez pas, car il s'agit la affections auxqnelles 
ou donne quelquefois, bien a tort, le meme nom et dont le prognostic est bien 
different." And again ; — " Sans doute, nosographiquement, les tic et la Choree 
representent bien, comme je vous l'ai dit, deux affections radicalement distinctes." 



LOCALIZED TIC. 73 

involuntary, bizarre, and much slower contraction of the muscles 
in chorea minor. 

As the varieties of tic pass insensibly into each other, it is 
difficult to make a satisfactory classification of the cases. I shall 
describe them in the following groups : — Simple tic, localized or 
general ; la maladie des tics convulsifs of the French ; the co- 
ordinated tics ; and lastly I shall speak of some forms of spasm of 
the respiratory muscles, as allied to the tics. 

I. — Simple Tic. 

Habit Spasm. Habit Chorea. 

The spasms may be localized or general. 

Localized Tic. — This, one of the commonest disorders of move- 
ment, begins usually in young persons, and may persist through 
life. The spasms are confined to a single muscle, a group of 
muscles, or a group of associated muscles. The muscles of ex- 
pression are most often involved, and in the common parlance of 
the profession tic means facial spasm. The idiopathic facial spasm 
of adults differs in many respects from habit spasm of the facial 
muscles. It is rarely seen until after the 40th year, is more 
common in women, the muscular contraction has not that quick, 
electric-like quality, but there are more often tonic and clonic spasms. 
The various forms of habit spasm are too well known to require 
description ; suffice it to say that the contraction may involve a 
single muscle, as the orbicularis palpebrarum on one side, or a group 
of the muscles of expression. Many forms are of trivial importance, 
but the more severe type, in which nearly all the muscles of the face 
are affected, and in which, during speaking, the depressors of the 
chin and the tongue muscles are thrown into action, constitutes one 
of the most distressing of the minor ailments of life. In many 
of these cases the affection seems to begin as a childish trick, 
particularly the blinking of the eyes, and the quick, rapidly repeated 
act of sniffing. Though not usually grouped with tic, the various 
forms of spasmodic wry-neck really belong to the same category of 
muscular disorders. The tic may be confined to the platysma 
muscle, as in a case which I saw a few months ago with Dr. H. M. 
Thomas. Habit spasm of the shoulder muscles is not uncommon, 
and is often a movement associated with a grimace or the act of 
winking. 

Simple tic is not so often seen in the muscles of the arms and 



74 CHOREA. 

legs, either in single muscles or in groups ; but in the former there 
may be quick movements of flexion and extension or of rotation, 
or of flexion and extension of the fingers. 

In the legs simple tic is less common. The most striking is the 
"springhalt tic." in which in walking at irregular intervals the leg is 
flexed rapidly on the thigh, sometimes with an associated movement 
of the muscles of one arm or of the face. 

The following is a remarkable case of long-standing tic of the 
muscles of the right leg : — 

Tic of the Muscles of the Right Thigh lasting for Thirteen Years, of 
late occasional Spasmodic Contractions of the Right Hand ; no 
Explosive Utterances. 

D. C., aged 44, lawyer, seen April 25th, 1893, complaining of a 
nervous twitching of the muscles, particularly those of the front of 
the right leg. He has always been a vigorous, healthy man ; comes 
of very good stock, and there are no nervous diseases in the family. 
He has been a moderately heavy smoker ; has taken alcohol daily ; a 
little more lately than usual, on account of worry about his condition. 

Thirteen or fourteen years ago, at a time when he was working 
very hard, he first noticed, in bed, just before going to sleep, that 
the muscles of the right thigh would twitch, and sometimes contract 
strongly enough to flex the thigh on the abdomen. At other times 
there would be a little tremulous creepy feeling beneath the skin of 
the thigh. It worried him very much and would at times keep him 
awake. There was no pain, but he was a good deal upset by it, 
and even dreaded the idea of going to bed. For a long time it was 
confined to the muscles of the thigh, but during the past few years, 
particularly if he had been very hard at work and much worried, a 
sensation would pass up the right side, and the arm and hand would 
jerk. Lately, if very nervous, or if hurried when writing, or if he was 
watched, the muscles of the hand would jerk a little, and once or 
twice the hand shook so that he had to stop writing. Otherwise he 
has been quite well ; there have not been any sensory disturbances, 
except an occasional uneasy feeling about the right leg or a little 
numbness or tingling in the right arm. Once or twice he has had 
vertigo. As a younger man he suffered a good deal from migraine, 
but has not had it at all lately. There have been no mental 
symptoms ; and no explosive utterances. The only acknowledg- 
ment of any special nervous sensation was sometimes feeling a 
little ill at ease and out of sorts when in company. 



GENERALIZED TIC. 75 

The examination was entirely negative ; there was no wasting of 
the muscles of the right leg ; sensation was perfect, and there were 
no changes in the reflexes. There was no indication of involvement 
of the nerves of the leg or of the spinal cord. 

The physical examination of the thoracic organs was negative. 
The patient was urged to quiet his mind on the subject, as he had 
had apprehensions that it would be a progressive trouble and cause 
paralysis, and told to rely rather on bathing and the cold pack 
before going to bed, than on the bromide and on alcohol. The 
twitching at night has evidently been serious enough to cause 
him a great deal of mental distress, and has been associated with a 
dread of going to bed and going to sleep, for which reason he has 
taken more bromide and alcohol than was good for him. 

Generalized Tic. — There is a very interesting group of cases in 
which the tic manifested in sudden, electric-like jerkings of the muscles 
of the trunk and extremities, causing a start which shakes the patient 
for an instant, and passing leaves him motionless and tranquil. The 
cases occur both in children and in adults. In the former the 
condition has been described as electric chorea 1 from the shock-like 
character of the movements. It is, however, not at all uncommon 
in adults, particularly in women, and the condition may persist for 
years. Very many of the cases have been described under the 
head of chronic chorea minor. The following are abstracts of 
histories of illustrative cases in adults : — 

For Four Years Electric-like Spasms of the Muscles of the Trunk 
and Limbs. Twitchings of the Right Corrugator Supercillii and 
Fight External Rectus. — Delusions. 

Susan B., aged 32, admitted November 24th, 1891, complaining 
of jerkings and general nervousness. The family history is good ; 
her father died of heart disease ; the mother and sisters have no 
nervous disorders. 

When eight years old she had typhoid fever, during which there 
was a transient speech disturbance. She grew to womanhood 
without any special troubles, and, with the exception of minor 
ailments, remained well until about four years ago, when she had 

1 The name Electric Chorea has also been given to Dubini's disease, probably 
an acute infection, met with chiefly in Lombardy, characterized by an onset 
with pains in the back and neck, and then contractions of the muscles, like those 
following electric shocks. They begin in the fingers, and spread over the body. 
Paresis of muscle groups occurs, and finally coma and death. 



76 CHOREA. 

acute Bright's disease with dropsy, from which she did not recover 
for a year and a half. During the convalescence the present 
trouble began with jerkings of the body and limbs, which have 
persisted ever since. She has never had any convulsions. Lately 
she has become suspicious of her friends, and has had delusions. 
The attacks, which come on at any time, consist in electric-like 
contraction of the muscles of the trunk, very abrupt and quick, 
passing off in a moment, and not moving her from the chair, or the 
place in which she may be standing. In bed, however, they will 
lift the back momentarily. Sometimes they succeed each ■ other 
rapidly for a minute or two, but she may pass an hour or part of a 
day without them. There are no facial grimaces and no movements 
of the hands or feet. After she had been under observation for a few 
weeks she began to have twitching of the right corrugator super- 
cillii, and the right external rectus contracted at intervals, causing an 
outward jerking of the eye-ball. On account of the increasing 
mental trouble the patient was transferred to an asylum. 

Electric-like Jerkings of the Trunk Muscles and Extremities for 
many Years ; Chronic Tuberculosis. 

A. B., aged about 27, was under treatment in Ward C for 
pulmonary tuberculosis of long standing. She had had for many 
years (she did not know how long) spasms in the muscles, some- 
times in those of the face alone, most frequently involving also 
those of the body and extremities. She had been treated by many 
physicians without avail, and had gradually learned to accept the 
condition as hopeless. Of late, since the onset of the lung trouble, 
the movements have been less marked. She was a small, delicate- 
looking woman, with chloasma, and bulbous fingers and toes. 
While talking the face muscles would occasionally twitch, par- 
ticularly those of the eyes, but the spasm was not at all excessive. 
At intervals of a few minutes or longer she gave an electric-like start, 
in which also the legs and arms seemed to participate, but she would 
not drop an article she was holding at the time. These jerkings 
did not disturb her very much, and she had become accustomed to 
them. They ceased during sleep. She was not hysterical. 

Sharp, sudden Spasms of Muscles of Trunk and Extremities of a 
Year's Duratmi, Epilepsy, Cessation of the Movements during 
Typhoid Fever. 

S. F., aged 21, admitted to ward G with involuntary jerkings of 



GENERALIZED TIC. 77 

the muscles and epilepsy. Her mother is of unsound mind. She 
has been well and strong, but for several years had had epileptic 
attacks. A year ago she began to have twitchings of the muscles 
of the body and limbs, and for this she sought relief. 

She was a well-nourished girl, a little dull-looking and pale. 
Every few moments there were quick, lightning-like contractions of 
the muscles of the arms and trunk, strong enough to lift the arms, 
but she would not drop objects from the hand. They were 
increased by excitement, and seemed worse when she was in bed. 
The legs also participated, but not to the same extent as the arms. 
In the intervals between the jerkings there were no twitchings. 
The face was not affected. The movements ceased during sleep. 
About sixteen days after admission she developed typhoid fever, 
during which the movements ceased entirely, to recur gradually 
during convalescence. 1 

Sprain of Ankle, Remarkable Choreiform Movements of the Trunk 
Muscles on the following Day. Neuritis of Nerves of Right 
Leg. 

Lieut. X., aged 31, in the service 10 years; always healthy and 
strong ; dyspepsia occasionally. 

Family history good ; father died at the age of 7 1 ; mother died 
in childbirth. Brothers and sisters well and strong ; no nervous 
troubles in family ; no history of any spasms or nervous troubles. 

He had not chorea as a child ; but since about the age of fifteen 
he has always been a little tremulous when excited. He has 
never noticed anything but this tremulousness. In 1886 he had 
muscular rheumatism after exposure ; pain in the shoulder and the 
legs below the knees. In October, 1890, he sprained the right 
ankle in jumping from a horse which was falling backwards. The 
ankle was bandaged, and he walked the next day with a stick ; but 
the joint was sore and swollen. Five or six weeks after he sprained 
it again, and the pain was so severe that he had to have a hypo- 
dermic injection. He has been lame ever since. The day after the 
ankle was sprained he had remarkable nervous twitchings, which 
seemed to be a jerking in the muscles of the trunk and abdomen. 
He seemed to feel as if he was under tension, and then, not being 
able to resist it, the jerking would come on. They were not pain- 
ful and not frequent, occurring only four or five times a day. For 

1 The case is fully described in the Report on Typhoid Fever, fohns Hopkins 
Hospital Reports, Vol. IV. 



78 CHOREA. 

three or four months they continued to trouble him, but at the Hot 
Springs he was better, and had no attacks. In New York, during 
the summer of 1891, while under treatment for wasting of the right 
leg, there was no return of the spasms. He returned to duty in 
October 1891. He was not able to do any riding, and gradually 
the nervous jerkings returned. They were not painful but were 
very unpleasant, as any one near by could notice them. He never had 
any jerking of the face muscles or of the hands. The spasms were 
confined entirely to the trunk muscles. There was no pain in the 
back at this time. He remained at the post until January 1892, 
when he was ordered to detached service with the Militia. He was 
very much better for a few months, though the ankle was still weak 
and painful. He was on duty until the 1st of November, and had 
no "jerking spells " except at the latter part of August. Altogether 
he was very much better, and in June was able to ride occasionally, 
and gave up walking with a stick. 

In November he was not so well, and the ankle troubled him a 
good deal. He was in Washington from November 1st until 
March 1893, under treatment most of the time for the back, which 
had become painful, and the leg. He had no jerking spells until 
February. In March he went to North Dakota, and there felt well 
until the 10th of April, when he had pain in the ankle, and the 
nervous jerkings recurred, and the riding caused pain in the back. 
The pains extended down the right leg, but were centred chiefly in 
the lumbar region. At one time they became so severe that he could 
not dress himself or turn. He was on the couch all day for a week, 
then was up and down for a couple of weeks, with occasional, but 
not severe, jerkings. 

From the 16th of May he has been in the house with a good deal 
of pain in the back, and when sitting up has had to have a pillow 
behind him. 

Present Condition. — He is a well-built, well-nourished man of six 
feet one inch in height, and looks very robust. When stripped he 
looks a man of fine physique, with well-developed muscles, but the 
right leg is decidedly smaller than the left. This is very evident on 
inspection before and behind. There does not appear to be any 
special difference between the thighs. The ankle is not swollen ; 
there is no wasting of the muscles of the feet ; pressure is a little 
painful at the posterior part of the inner malleolus, and for a couple 
of inches in this region in the course of the posterior tibial nerve. 
The patient can perform all the movements, and there is no weak- 



GENERALIZED TIC. 79 

ness of any special group of muscles. The lumbar muscles are 
equal in volume, and the spine is straight. All the twisting and 
bending movements of the back are performed without any pain. 
He complains of a little pain and tired feeling in the lumbar region, 
and there is a little sensitiveness on deep pressure in the course of 
the right sciatic nerve. There are no sensory disturbances in the 
area of distribution of the sciatic nerve. 

The knee-jerks are normal ; not exaggerated on either side. 

Examination of the other organs is negative. The pupils are 
equal, and respond to light. 

The patient does not give one in any way the idea of a neuras- 
thenic subject. 

He remained under observation for about two months, and with 
massage and electricity the local conditions improved rapidly. The 
jerkings, as he calls them, occurred now and then. I only saw them 
once, when a sort of exaggerated shiver seemed to pass through 
him, and moved the trunk and shoulders, but not the head or 
extremities. He says they were very transient and went through 
him like a flash. 

II. — Tic with Coprolalia, Echolalia, &c. 
{Maladie de la Tic Convulsif. Gilles de la Tourette's Disease.) 

In the second group may be placed the cases which present, in 
addition to the motor disturbance, certain remarkable features, as 
the explosive utterance of certain words or sounds, and a mental 
state characterized by the existence of fixed ideas. Particular atten- 
tion has been paid to this form by Charcot and his pupils, and it 
has been made the subject of a special memoir by one of them, 
Gilles de la Tourette, 1 whose name is now often given to the affection 
in France. 

The following cases will illustrate fully the chief features of the 
affection. 

Case I. — Tic for Eight Years; Coprolalia; Echolalia. 

Mary , aged 13 years, applied at the out-patient department 

Johns Hopkins Hospital, July 10th, 1890, and was under observa- 
tion there until September 16th, when she was admitted to ward G. 

1 Archives de Neurologie, 1885. In the Lecons du Mardi of Professor Charcot, 
1887-88, pp. 65, 105, 294; and 188S-89, pp. 13, 464, will be found a full discus- 
sion on many interesting cases. 



80 CHOREA. ■ 

Her mother brought her to the hospital on account of irregular 
involuntary movements and curious barking sounds. 

The family history is good. Her mother is a bright, intelligent 
woman, a German by birth, who has had ten children, none of 
whom have been affected as is this girl — the third child. There is 
no tendency to mental disease in the family. The birth of the child 
was normal and there is no history of convulsions in infancy. She 
has had scarlet fever, but has not had rheumatism. 

Since her fifth year she has been subject to involuntary jerking 
movements of the arms and head, which vary very much in intensity, 
sometimes better, sometimes worse, and they have usually been 
called by the doctors chorea. They have not interfered with her 
development or her education. She has not yet menstruated. For 
the past year she has been making curious sounds ; beginning by 
saying "hah" very frequently. Sometimes she would bark like a 
dog. She would also call out the names of people, and if she heard 
a new name she would be apt to repeat it. 

Her condition on admission was as follows : — A bright, intelligent 
child ; well educated, writes nicely, takes an interest in her books 
and has evidently been ambitious at school. The right arm occa- 
sionally twitches and the head jerks. There are no grimaces, but 
on several occasions she seemed to mimic movements of the face. 
Every now and then she calls out " hah," " Bridget," or " stools " ; or 
says in sharp, clear tones, " bow, wow." There are no disturbances 
of sensation, and the special senses are unimpaired. Examination 
of the heart and lungs is negative ; the thyroid gland is slightly 
enlarged. 

Throughout the latter part of July and August attempts were made 
to treat the case by hypnotic suggestion, at first with success, but 
subsequently without any improvement. 

On September 8th her mother wrote the following letter, which 
illustrated a new phase of the child's malady : — 

" Mary makes use of words lately that make me ashamed to bring 
her to you, or to take her out of the house ; it is dreadful ; such 

words as , , , etc. She was always a modest child, 

and it almost kills me for to hear her use such words." 

Her mother was asked to bring her again, and was told that this 
was really a part of the affection, and, like the movements, involun- 
tary in character. The child seemed more depressed, had lost flesh 
and, her mother said, had changed mentally. She was very obstinate, 
and almost invariably did what she was told not to do, and had 



TIC WITH COPROLALIA, ECHOLALIA, ETC. 8 1 

threatened to take poison. She will say the bad words aloud or 
mutter them to herself. 

On admission to the hospital she was placed in a room by herself, 
kept in bed, and encouraged in every way to cease making the 
sounds and to stop the use of the bad words. During the first two 
weeks she improved very much. The movements were reduced in 
frequency, and sometimes during my visit they would not be noticed 
at all. . They most commonly affected the right arm, which, with 
the hand, was drawn up in a sudden electric-like jerk. The head 
and neck would jerk simultaneously or alone. Sometimes there was 
combined movement of the neck and chest-muscles. The involun- 
tary expressions of which she made use were those mentioned above ; 
a sharp bark was the most frequent sound, which, from its ringing 
quality, could be heard at a considerable distance. 

She improved and was allowed to get up, and another patient was 
placed in the room with her. This seemed to excite and worry her, 
and shortly afterwards the barking sounds became much more fre- 
quent, occurring every one or two minutes, and she complained of 
great soreness of the muscles of the chest and abdomen. The 
movements, however, did not increase. She was again placed in 
seclusion and in bed, and again improvement followed, but she still 
barked and did not give up entirely the use of bad words. 

She is a docile, intelligent child, and seems anxious to get well. 
She has kept a diary, which displays no special peculiarity. She 
writes verses, which are not worse than those usually composed by 
girls of her age. 

Case II. — Tic of the Muscles of the Face and Neck ; Fixed Ideas ; 
Arithmomania. 

A. B., aged 13, seen Sept. 6th, 1890. She is an only daughter in 
a family with marked neuropathic taint. The father died insane ; 
the mother is a high-strung, nervous woman. 

The child is well grown, and well nourished, though rather stout 
for her age. She is very bright and intelligent, and perhaps has not 
had as much control as was good for her. For a year or more she 
has had occasional twitchings of the muscles of the face and neck, 
noticeable in the quick sudden elevation of the eyebrows, or in 
movements of the platysma muscles. They have not been severe, 
and for days it may not have been at all noticeable. There have 
been no spasmodic movements of the arms or legs. The condition 
has not interfered in any way with her growth or development. She 

G 



82 CHOREIFORM AFFECTIONS. 

is very fond of outdoor exercise, particularly of riding on horseback. 
A short time after the onset of the twitchings it was noticed that 
she began gradually to have all sorts of queer notions and practices, 
many of which persisted for some weeks or months, and were then 
changed for others not less anomalous. Some of her vagaries are as 
follows, nearly all being modifications of the fixed idea known as 
arithmomania. Before getting into bed at night she lifts each foot 
and taps nine times on the edge of the bed. After brushing her 
teeth she has to count one hundred. For a year at least she has 
always entered the house by the back door, protesting that she 
never can enter by the front door again. Lest her mother should 
prevent her getting in by the back door, she for months carried the 
key herself. On reaching the door she knocks three times on the 
edge of the window near by, and three times on the door before 
unlocking it. She will not under any circumstances button her 
shoes. For a long time she would not pronounce the name of 
anyone, but would spell it, and if she wished for anything at the 
table she would spell the word, but not pronounce it. In drinking 
water she will take a mouthful, then put the tumbler down, turn it 
once or twice and repeat this act every time she drinks. She would 
not brush her hair except at the extreme tips, and it is only under 
the strictest compulsion that she will allow the hair on the top of 
the head to be combed. Before putting on clean under-clothes she 
has to count so many numbers that there is a great difficulty in 
getting her to make the change, except under the strongest threats 
from her mother. 

The patient was sent to the country under the care of her aunt, 
who was urged to control and train the child. 

A special interest attaches itself to this case, inasmuch as the 
patient has recovered completely. I have seen her on several 
occasions within the past three years, and she has grown into a fine 
young woman ; both the tic and the mental symptoms have dis- 
appeared. 

Case III. — Convulsive Tic, with Echolalia, Coprolalia, and Delire 

du Toucher. 

Delia L., aged 12, sent by Dr. Goldsborough of Cambridge, 
Md., complaining of involuntary movements and of loss of control 
over speech. 

Family History. — She comes of good stock without neurasthenic 
taint. Father and mother are living and well. The mother seems 



TIC WITH COPROLALIA, ECHOLALIA, ETC. 83 

a sensible woman and is not at all nervous. There are two sisters 
and two brothers living and healthy. 

Personal History. — She has been very healthy ; she has had 
measles and whooping-cough ; but neither rheumatism nor muscular 
pains. She is of an excitable temperament, and very bright mentally 
and ambitious at school. She gets very excited at play, and lately 
has been using a tricycle. 

About a year ago the mother noticed that she winked her eyes 
quickly at times, and had jerking movements of the head. As they 
were slight at first not much attention was paid to them. Subse- 
quently she had sudden, quick twisting movements of the body. 
The arms did not jerk, but she had occasional jerking movements 
of the legs. Since the summer the movements of the head and 
trunk have increased a good deal, and come on at intervals of a few 
minutes or so, and are much aggravated by excitement. She con- 
tinued very well physically. 

In August, 1893, a new feature developed; she would at intervals 
shout out words of all sorts without being able to prevent it, partic- 
ularly such words as "murder," "fire," and at times obscene words 
which she picked up from hearing boys use them in the street. These 
would sometimes be called out very loudly with an associated move- 
ment of the head. She also repeated words or short sentences 
which she heard, and after reading a street sign, she would often 
call it out in a loud voice several times, or if she met a person and 
heard his name she would repeat it loudly. About the same time 
she began to touch objects, and would stoop down and touch the 
floor, or touch certain things in the room, particularly the lamps. 
She also would frequently stoop down and knock with her knuckles 
on the floor a certain number of times, or would rap on the table. 

Her general condition has kept good. She has often been 
excited and wayward, but her appetite is good and she sleeps 
well. 

Present Condition. — She is a well-grown girl for her age. The 
hair and eyes are dark, and the expression very bright. She sits 
quietly for a time and talks without any embarrassment and quite 
readily. Occasionally she will wink rapidly with her eyes, and 
elevate the eyebrows. Every few minutes the head jerks with a 
sudden, quick, lateral, and forward movement, evidently with a 
contraction of the muscles of the thorax as well, as the trunk also 
moves slightly. These occur at irregular intervals, and are rather 
more frequent under excitement. Every few minutes there is a 

G 2 



84 CHOREIFORM AFFECTIONS. 

sudden explosive utterance. During the examination there was no 
special word, but an indefinite sound, often quite loud. Frequently 
during the examination she would touch objects, apparently without 
self-consciousness ; thus she would stoop and touch the floor, and 
she touched on several occasions the tip of the pen with which 
Dr. F. A. Smith was taking notes. There were no movements of 
the hands or of the legs. 

The physical examination was negative. There were no sensory 
disturbances, and the reflexes were normal. 

Case IV. — Tic with Subjective Auditory Hallucinations. 

Eliza D., coloured, aged 53, seen January 13th, 1894. She is a 
married woman ; has been healthy and well as a rule, and so far as 
can be gathered has had no serious illnesses, or any mental dis- 
turbances until the onset of her present trouble. The menopause 
occurred five years ago. At this time she was much troubled with 
hot and cold flushes and began to have a twitching of the left 
shoulder, and it is for this, which has continued, that she seeks 
relief. 

Patient is a well-nourished woman, a little excited and garrulous. 
Every few minutes the left shoulder is lifted and the head drawn to 
the left. The movement is rather slow and does not affect the arm 
and hand. When she is standing up it evidently involves the trunk 
muscles, as the body is rotated and makes a half turn as the 
shoulder is drawn down. This may occur without any rotation of 
the head, but sometimes they occur together. The twitching has 
continued almost uninterruptedly, and, she thinks, has of late become 
aggravated. But what troubles her most seriously are certain 
sounds which she hears, as if people were talking to her. She 
never herself makes any explosive utterances, but during the 
movements she hears distinct sounds or noises, which she says 
are only present when the movements occur. It is impossible to 
get a good account of the nature of these noises, whether they are 
words or merely sounds. Some time ago she had also hallucinations 
of sight, but these have not been present lately. 

The special features of the affection may now be considered. 

(a) The Involuntary Movements. — These vary from trivial, 
slight spasm of the facial muscles, as in Case II., simple tic, to the 
most extraordinary bizarre movements, involving all the muscles of 
the body. The muscles of the face are most commonly affected, 
and there is a sudden, rapid closure of the eyelids, elevation of the 



INVOLUNTARY MOVEMENTS. 85 

eyebrows, or a quick, lightning-like movement of the muscles of 
expression, producing an unnatural-looking smile. As in simple 
habit spasm, a somewhat frequent form is the quick, rapid sniffing 
of air. Instances have been described, too, in which there were 
rapid movements, alternately opening and closing the mouth. The 
chin may be suddenly depressed, and a frequent movement is the 
rapid contraction of the platysma, moving the skin of the neck and 
lower part of the face and over the shoulder. The movements may 
be limited to the face, one side or the other, but more commonly 
other muscles are affected, and a sudden spasm of the sterno- 
mastoid may draw the head quickly to one side, and at the same 
time there is a facial grimace. A not uncommon movement is a 
sudden jerking upward of the head, with a rapid lateral motion- 
Affection of the muscles of the shoulder girdle is common on one 
or both sides, and with or without movements of the arm. The 
movements of the upper extremities are very varied. There may 
be, as in Case IV., with rotation of the trunk, a drawing backwards 
of the arm ; or, as in Case III., slight jerking movements, some- 
times of pronation and supination, or of flexion and extension. 
Other movements may be made of a purposive nature ; thus the 
movements, as in Case III., of definitely touching an object, which is 
common, or of scratching any part of the body, or the hand may 
be frequently placed before the mouth as in coughing, or both hands 
may be rubbed together as if in great glee. 

Isolated movements of the trunk muscles are not so common, but 
the salaam movements may be present in this affection. 

Isolated movements of the lower extremities are rare, but the leg 
may be abruptly extended, or, what is more common, there is 
either, as the patient stands or walks, a sudden spring-halt, due to 
contraction of the flexors of the legs and of the thigh. 

And lastly, there are instances on record in which the movements 
are of a most unusual and astonishing character ; thus a patient 
whom I saw at the Salpetriere, and whose case is described by 
Gilles de la Tourette, would run rapidly for a short space, then 
touch the knee, and perform the most remarkable movements with 
the arm. As a rule the patients have no difficulty whatever in 
carrying out the movements of the will, that is, the ordinary 
voluntary movements. They write, feed themselves, etc., without 
any trouble, though sometimes in a patient affected with serious 
tic of the arm there may be some difficulty in writing. 

The movements are much influenced by emotional causes ; thu 



86 CHOREIFORM AFFECTIONS. 

in the presence of strangers, and when under observation, as during 
the examination, they may be greatly exaggerated. When the 
patients are quietly at home, or isolated under treatment, the 
movements may be quickly reduced. In some instances any 
sudden sound may at once cause a series of rapid irregular con- 
tractions. They cease always during sleep, and in several cases it 
has been noted that an intercurrent fever has checked the move- 
ments entirely. In some instances a powerful exercise of the will 
may restrain to a great extent the movements, but in young 
children the anxiety in a voluntary attempt to restrain them some- 
times causes an aggravation. 

There are curious associated actions with the tic ; thus a lad 
under my care at the Infirmary, who had facial tic, was in the 
habit of rapidly grasping the middle finger between the teeth, 
biting it quite hard, and at the same time pressing with the 
index-finger the tip of the nose. So frequently had the action 
been performed that thick callosities had been produced on the 
skin of the second phalanx of the middle finger. Such actions are 
difficult to separate from the simple tricks which children practise, 
as in the case of Hartley Coleridge, who when a boy was in the 
habit of biting his arm ; and I remember seeing an instance a few 
years ago of a young girl recovering from chorea minor (?) who 
when she took anything into her hand had the curious trick of first 
smelling and then blowing upon it. 

(b) Involuntary Cries. — Coprolalia. — Echolalia. — Cases I. 
and III. illustrate very well the character of the explosive utterances 
which form so characteristic a phenomenon in this affection. These 
may be nothing more than the exclamation of "ah !" "ahem !" or 
"oh !" associated in each instance with an involuntary movement. As 
in Case I. the same may have a somewhat barking quality, two sounds 
succeeding each other quite rapidly. In Case III. there was the 
involuntary utterance of all sorts of words, such as " murder ! " " fire ! " 
or any name which she had heard, and which seemed to take her 
fancy, would be repeated throughout the day. Not only would the 
girl repeat words, but short sentences, and after reading a sign on 
the street she might repeat it aloud many times. 

A remarkable feature, in some instances, is the irresistible tendency 
to repeat words or names which have been heard, and which Gilles 
de la Tourette has designated by the term echolalia. The word or 
phrase which the patient has heard may be frequently repeated ; 
thus on the day on which Case I. was brought to the hospital, she 



INVOLUNTARY CRIES. 87 

heard the word nurse frequently, and repeated it for some time. 
The patient may often echo words which she has said herself. A 
distressing feature, and which gives to the affection one of its most 
annoying characters, is the habit which the patients have of using 
bad words, designated by the word coprolalia. Though these are, 
as a rule, involuntary, yet the child may be to a certain extent 
restrained by the presence of strangers ; thus in Case I. the girl was 
in the habit at home of using shocking words, to the great distress 
of her mother, but during examination, and, indeed, during her stay 
in the hospital, she very rarely employed them. In Case III. this 
feature of the malady gave the greatest possible distress to the rela- 
tives. Thus with a shrug of the shoulder, or with a facial grimace 
a young child may horrify her parents or those about her by saying 
" God damn," or " Jesus Christ," or using words of the most obscene 
character ! They are often used on the street, and may sometimes 
get the poor victim into difficulties, as in a young lad, whose case 
is reported by Gilles de la Tourette, who called out with his tic, 
conillon. He was thought by some other boys to be addressing 
them, and the poor coprolalic was given a good thrashing. 

Not only may the patients mimic or echo words, but certain 
movements may also be mimicked at once, to which habit Charcot 
has given the name echokinesia. Any movement which is made is 
at once imitated, whether it be a facial grimace or a movement of 
the hands or of the body. This feature is marked in the affection 
known as Latah, seen among the Malays, and which, with the 
jumpers of Maine and the Myriachit of Russia, appears to be a 
variety of tic. 

{c) Many cases present remarkable mental features, which, with 
the explosive utterances and coprolalia, complete the picture. They 
come under the group of the imperative ideas, 1 obsessions mentales of 
the French. These fixed ideas, as they are also called, may be a 
very troublesome manifestation, taking very varied forms, among the 
most common of which is the agoraphobia, or the dread of walking 
in a large open space ; the folie fiourquoi, or insanity of doubt, in 
which a patient incessantly demands the reason for the performance 
of even the simplest acts of every-day life ; the onomatomania, a 
curious mental state, in which a word or name constantly recurs, and 
the impulse to repeat it is irresistible. Case II. illustrates well the 
obsession known as arithmomania. Before attempting to perform 

1 For full discussion of these, see Article by Dr. D. Hack Tuke, Brain, summer 
number, 1894. 



88 CHOREIFORM AFFECTIONS. 

the common, every-day actions of life, this child had the irresistible 
impulse to count a certain number of times, or had to tap a certain 
number of times with her hand or foot. Another common manifesta- 
tion is the imperative impulse to touch certain objects. This was 
particularly well seen in Case III., and in her it seemed to be 
almost a spontaneous unconscious action. This delire du toucher 
in children is often a mere trick, and may persist to later life, as 
in the well-known case of Dr. Johnson. In adults, associated 
with forms of tic, it constitutes one of the most interesting forms 
of street pathology; thus, I have seen a man who, as he walked 
along, would go beneath the windows of a house, swing his hand 
slowly two or three times, and then touch a portion of the window. 
It is a very common trick in boys, and usually of no special moment. 
George Borrow makes use of it in an interesting manner in his 
" Lavengro," describing his imperative impulse to touch the top of a 
certain tree. In his case it was associated with an idea that the 
performance of the action would ward off some evil to his mother. 

The prognosis of this affection is very uncertain. Usually the 
cases last for many years, and Charcot and his pupils regard the 
outlook as very unfavourable, Guinon stating that it is, as a rule, 
incurable. In Case I. the condition had lasted for eight years, and 
she did not improve at all under seclusion and treatment in the 
hospital. Case II., on the other hand, made a complete recovery, 
and both the arithmomania and the involuntary movements have 
disappeared. The coprolalia may persist an indefinite time, as in 
the case of the Marquise de Dampierre, who involuntarily used 
shocking language on most inappropriate occasions from his earliest 
youth to the age of ninety. 

III. — Complex Co-ordinated Tics. 

Under this heading may be grouped a number of forms 
of habit movements, differing from ordinary tic in the more complex 
character of the action performed, which may be one of every-day 
life, but one which is repeated without obvious cause and which 
can be controlled or arrested by an effort of the will. 

(a) Many tricks and habits are of the nature of this co-ordinated 
tic, as the action of a distinguished President of the Royal College 
of Physicians, who in writing stopped at every few words and looked 
intently at his finger tips. The tricks of children, head-nodding 
(though not always), thumb-sucking, and rocking in bed (a habit 
which may persist to adult life), are allied actions. Some of these 



COMPLEX CO-ORDINATED TICS. 89 

forms I have already mentioned in the last section, particularly the 
case of the child recovering from chorea minor (?), who on taking 
anything in the hand first smelt and then blew upon it, and the boy 
with facial tic, who bit his finger hard and pressed his nose at the 
same time with the index-finger. 

Possibly the interesting affection of children known as head- 
nodding, and which has been described so fully by Gee, 1 Hadden, 2 
and Peterson, 3 belongs in this group. In a case at the Infirmary for 
Diseases of the Nervous System, a child, aged two-and-a-half years, 
would sit on the floor by the hour, playing with a few toys, and 
nodding the head every few moments ; not a simple up-and-down 
motion, but with a rotation from left to right and right to left. The 
child had also lateral nystagmus and was feeble-minded. Some of 
the cases have followed injury, usually in early life, but not at 
birth. The nystagmus and the feeble-mindedness point, in some 
cases at least, to organic changes, yet both Gee and Hadden 
state that the prognosis for recovery is fair. The condition is to be 
differentiated from epilepsia nutans in children. 

Another strange disorder of allied nature is the " head-banging " 
in children, of which cases are described by Gee in the same volume 
of the Bartholomew Hospital Reports. In one case, a child of 
five, the habit had lasted two-and-a-half years. Asleep or awake, while 
in bed, the child would turn over and bang the head violently into the 
pillow, repeating the act five or six times. In another case the act 
was repeated for two or three hours at a time. No other symptoms 
were present. In one of the cases a younger brother also caught 
the trick. 

(b) An extended series of these co-ordinated movements are met 
with in feeble-minded children. Balancing, rotation of the head 
from side to side, and the striking of the chin violently against the 
chest are extremely common habits. The repeated beating of the 
forehead or the face with the hand or fist is another trick, frequently 
seen in imbeciles, to which Rubinowitch has given the name of 
Krouomania. Many of these movements are rhythmic, particularly 
the balancing, the nodding, and the rotation. Noir, 4 from 
Bourneville's division of the Bicetre, has described many cases of 
tic of various kinds in the blind, particularly the rapid movements 
of the fingers before the eyes. In other instances the movement 
may be one of jumping, in which the patient makes a series of leaps. 

1 St. Bartholomew's Hospital Reports, Vol. XXII. 2 Lancet, 1890, I. 

3 Medical News, 1892, I. 4 Etude sur les Tics. Paris, 1893. 



90 CHOREIFORM AFFECTIONS. 

In others, again, he performs in orderly sequence a series of actions, 
such as stooping from the chair, lying prone on the floor, raising the 
hands above the head, &c, all of which are repeated from time to 
time. 

At my visits to the Institution for Feeble-minded Children at 
Elwyn, while studying the hemiplegic and epileptic cases, one room 
in the Hillside division had always a special attraction. In it was a 
remarkable collection of the unhappy victims of " irresistible 
musculation." One had not crossed the threshold before the hand 
was grasped by a bright-eyed, well-knit little fellow, sharp and active 
as a fox-terrier and restless as a wolf, an imbecile, with the motor 
centres abnormally active, and with a facial tic, displayed in a 
lightning-like contraction of the muscles of expression. Hemiplegics 
with mobile spasm, poor William B., a case of double athetosis, and 
epileptics, apparently engaged our attention, but we watched in 
reality a curious Astec-like idiot, known as the " Dervish." In a few 
minutes he would rise from the floor, balance for a moment, take two 
or three gentle, sweeping rotations, then poise himself and begin a 
series of the most extraordinary gyrations, moving but slightly from 
one spot. At first the rotatory movement was slow, and readily 
followed, but soon the speed increased, and with arms out and clothes 
flying, and form and features almost unrecognizable, he span round 
like a humming-top until he dropped exhausted. 

{c) In this group of co-ordinated tics may be placed those cases 
of extraordinary and bizarre movements repeated at intervals for 
perhaps a long period of years, sometimes, but not always, associated 
with imperative ideas or explosive utterances, as described in the 
preceding section. The remarkable case reported by Weir Mitchell 1 
of a man who for years had a sort of pendulum spasm, in which, 
unless at perfect rest in the recumbent posture, the left arm would 
strike the side in a regular order, at the rate of from 150 to 160 times 
in a minute, is an exaggerated example of this form of tic. 

IV. — Cases of Spasm of the Respiratory Muscles. 

There is a series of cases in which a recurring spasm affects the 
muscles of respiration and phonation, and the muscular contraction 
is accompanied with more or less noise, either a sniffle or hiccough 
during inspiratioti, or some noisy utterance or explosive sound 
during expiration. These constitute the two varieties. While 

1 American Journal of the Medical Sciences, 1876, II. 



SPASM OF THE RESPIRATORY MUSCLES. 9 1 

many of these cases are hysterical, in others no features save the 
respiratory spasm are met with. 1 The following are illustrative cases : 

Case I. — Loud Inspiratory Noises following a Fit of Anger. 

Rebecca T., a Russian girl, 20 years of age, came to the 
dispensary on November 5th, complaining of what she called hic- 
cough. 

As regards her family history no neuropathic tendencies could be 
traced, and she herself has shown no special signs of nervousness. 
Excepting an attack of typhoid fever two years ago she has always 
been a healthy and strong girl. Her menstruation has been regular 
to October of this year. A year ago, following some family trouble, 
she left her home and came to this country. 

Her present disease began shortly after a fit of anger about the 
middle of October. She then began to produce peculiar spasmodic 
sounds, which at times she utters in quick succession, while at 
others she remains perfectly quiet for as long as two or three hours. 
She has also had crying spells. For some years she has had severe 
headaches, which, since the onset of the present illness, have 
become more intense and more constant In the waiting-room of 
the dispensary she was quiet, and had been so, we were told, on the 
street. Immediately upon entering the examining room, however, 
she began to produce these gasping sounds. 

The following note was made in the ward : — The patient is a well- 
nourished girl, not anaemic, and there is no fever. The examination 
of the thoracic organs is negative, and nothing abnormal is detected 
in the abdominal viscera. There are no tender points along the 
spine. Patellar reflexes are quite active ; they may even be called 
exaggerated. The most striking peculiarity about the patient consists 
in the production of loud, gasping noises, occurring during inspira- 
tion and often prolonging it. They come on at irregular intervals, and 
are usually quite loud, but vary in intensity and frequency, increas- 
ing in both respects during observation. Expiration is normal, and 
during it no sounds are ever produced. During sleep they are 
absent and respiration is quiet. Sometimes the sounds bear a 
resemblance to a hiccough. During the crying spells she rarely 
shed tears, but merely produced sounds in rapid succession which 
resembled sobbing. If the door is suddenly opened the noises at 
once begin, while they may be absent for some length of time 

1 The best discussion on the different forms is by Charcot in the Archives de 

Neurologie, 1892. 



92 CHOREIFORM AFFECTIONS. 

during quiet conversation, even if she has been extremely noisy 
immediately before. Sometimes the sounds are short and produced 
in quick succession, or six or more long-drawn, very loud inspiratory 
sounds may follow each other. During her stay at the hospital a 
rise of temperature was noticed, especially on the first, third and 
fourth days, reaching ioo° twice, once 99 '5°. 

In this case hysteria was very probably the underlying condition, 
though there were no disturbances of sensation or of sight. In a 
very similar case in the wards last winter the hysterical characters 
were pronounced. 

It is difficult sometimes to determine the exact origin of these 
explosive sounds ; thus, in the following case the patient insists that 
the noise which she produces is preceded by a rumbling roll very 
low down in the abdomen, and both she and her sister speak 
of it as noisy belching, but from the character of the noise which 
she describes it is more likely to be produced in the respiratory 
passages. 

Case II. — Noisy Explosive Sounds. 

Miss R, aged 24, seen with Dr. Bosley, May 23rd, 1893, com- 
plaining of a very remarkable noise which she makes at intervals. 
The patient has always been well and strong. She has had 
measles, scarlet fever, and whooping-cough. Eight years ago after 
an alarm of fire in a theatre she lost her voice for three months. 
She has, however, enjoyed very good health and has not been 
specially nervous. She has not had hysterical spells ; i.e., of laugh- 
ing and crying, and has not been very emotional. She lost her 
father a year ago, and since that time she has been living a very 
quiet life. Her mother is an exceedingly quiet, reserved woman ; 
not at all nervous. The other members of her family are perhaps 
a little excitable and nervous. 

About the last of March, one Wednesday evening, hearing a 
knock at the door, and expecting some girl friends, she ran to open 
it. She had a " caramel " in her mouth at the time. Instead of 
finding the persons she expected there was a young man of her 
acquaintance, and not liking to be found with her mouth full, she 
swallowed the bon-bon suddenly. She felt no bad effects at the 
time, but that night had cramps in the abdomen. These continued 
the next day, and she had diarrhoea and passed a little blood. The 
following night too she had quite severe cramps. Evidently, from 
what her sister said, she was a good deal alarmed and uneasy about 
herself. On Saturday morning she began to make a remarkable 



SPASM OF THE RESPIRATORY MUSCLES. 93 

noise, something like a belch, but very much louder, and this has 
persisted ever since. She describes it as a rumbling roll which 
comes from low down in the abdomen, passes up the stomach and 
comes out at her mouth as an explosive loud noise. She can tell 
beforehand by uneasy feelings in the abdomen, but finds it impossi- 
ble to stop its onset. She can put her handkerchief quickly to the 
mouth and deaden the noise. It occurs from sixteen to eighteen 
times in the day. It is more frequent in the morning after she gets 
up. It is much less when she is at rest, but turning in bed is apt to 
produce it. No fluid comes up with it ; sometimes a little acid. 
Occasionally there are two or three smaller sounds following the 
first loud explosion. The sister describes the noise as " terribly " 
loud, and it can be heard several rooms away ; frequently on the 
street it is enough to make people start. Diet does not appear to 
have much influence. It has been a good deal worse lately on 
peptonized milk. Curiously enough it is not worse when she is in 
company, or not rendered worse by emotion. Thus her doctor has 
never heard it, and in the half-hour which she spent at the consulta- 
tion with me the sound was not once made. 

The patient is a well-built, medium-sized young girl, looks bright, 
and the colour good. She has, however, lost five or six pounds, and 
her sister says she does not look at all well. The physical examina- 
tion is entirely negative ; there are no other hysterical features in 
the case ; the stomach is not dilated. During the examination she 
stated that frequently the abdomen would swell and become too 
tight for her waist-band. The right kidney is not palpable. 

She was so insistent that the sound came from the abdomen 
that I advised her to have the stomach washed out once or twice a 
week, but it had no influence, psychical or otherwise. 

Case III. — Tic of the Deglutition Muscles, with a slight Clicking 
Noise ; subsequently Facial Tic. 

Laura H., aged n, a very bright-faced, nervous-looking child, 
applied at the Dispensary April 5th, 1894, complaining of a curious 
movement in her throat. An uncle had Parkinson's disease, and 
the mother had chorea. The child has had typhoid fever, chicken- 
pox, and measles. 

When three years old she had an operation for strabismus per- 
formed. She is a very bright girl, and is advanced at school. 

Two weeks ago her present trouble began quite suddenly with a 
peculiar motion of her throat, which continues constantly during the 



94 CHOREIFORM AFFECTIONS. 

day, and disappears only when she is asleep. She has no other 
twitching, and complains of nothing else. At first there seemed to 
be a peculiar noise in the throat, which has, however, now almost 
ceased. There is no history of any explosive utterance. 

On the 6th I made the following note : — A bright-eyed, clear-cut, 
intelligent-looking child ; no movement of facial muscles ; slight 
strabismus of left eye. At first nothing was noticeable, but in a few 
moments rhythmical movements began in the neck, which were 
practically those made in deglutition, and associated with elevation 
and depression of the hyoid bone, and of the thyroid cartilage. 
These parts are drawn up and down rhythmically, causing a wavy 
movement beneath the skin, such as occurs in the act of swallowing. 
They are repeated sixty- eight times in a minute. Drinking water 
makes no apparent difference. During the movement there can be 
felt a sort of click, and when she holds her head back a slight noise 
is heard, also like a click, as though the cartilages were rubbing 
over one another. The heart-sounds are somewhat rapid, and the 
first is reduplicated at the apex. 

The movements in the throat gradually ceased. I saw her 
June 26th, and she said she had not had them for two weeks, but 
she has now a well-marked facial tic, involving the muscles of the 
eye-brows and the right orbicularis palpebrarum. 

Various forms of this respiratory spasm are in reality quite 
common. Terribly exaggerated instances, among the most dis- 
tressing of all afflictions to the friends and relatives, are encountered. 
On the 30th of June, 1884, I saw brought into Professor Wagner's 
clinic at Leipzig a most remarkable case of this kind ; a young girl, 
aged fifteen, who had always been healthy, but who had worked 
very hard at school. Some time about the end of March she awoke 
one night with difficulty in breathing, and ever since has had the 
remarkable respiratory spasms and cries to be described. They 
have persisted the entire day, ceasing only with sleep, and as the 
child has taken but little food, she has wasted to a skeleton. Before 
the patient was wheeled into the clinic, the noises which she 
made could be heard at a great distance. They consisted of a loud 
and very intense inspiratory cry, sometimes preceded by three or 
four short, jerky inspirations. Then following the cry there was a 
deep, hoarse, very loud expiratory sound. These followed each 
other with remarkable sequence, the child sitting up in bed and 
swaying to and fro. The examination of the lungs was negative ; 



SPASM OF THE RESPIRATORY MUSCLES. g$ 

there was, of course, extreme dryness of the throat. I abstract the 
following from the notes which I made on the occasion : " Professor 
Wagner put his thumbs in the child's mouth and depressed the 
lower jaw forcibly, and urged her to breathe quietly and gently. 
The movements ceased almost immediately, she took a deep 
inspiration, lay back in the bed, and breathed quite quietly, told 
her name, and said that she had been much overworked at school. 
She took a glass of water and seemed quite quiet and natural." I 
did not hear the subsequent history of the case. 

A few years ago Dr. Gapen of Omaha brought a phonographic 
cylinder on which was recorded a cry of a somewhat similar nature, 
uttered by a young girl for many months. It was loud enough to 
be heard at a distance of several city blocks. 

No doubt the ptussis canina of old writers, in which the patients 
uttered short explosive sounds like the bark of a dog, though in 
many cases an expression of hysteria, belongs in this category • 
possibly, too, some of the instances of the barking cough of 
puberty.. 



g6 CHOREA. 



CHAPTER VII. 

CHRONIC PROGRESSIVE CHOREA. 

Huntington's Chorea. 

Historical Note — Report of two Families— Special Symptoms — Morbid Anatomy 

— Diagnosis. 

Historical Note. — The affection was first described by physicians 
of the State of New York, in parts of which it appears to have 
existed for very many years. In the first edition of Dunglison's 
"Practice of Medicine," published in 1842, on page 312 of the 
second volume, he gives a letter from Dr. C. O. Waters, of Franklin, 
New York, in which an affection is described as prevailing in the 
south-eastern sections of the State, where it was known as the 
" megrims " — " consisting of a spasmodic action of all or nearly all 
the voluntary muscles of the system — of involuntary and more or 
less irregular motions of the extremities, face, and trunk." The 
throat muscles sometimes participated, as evidenced by the clucking 
sound. The disease was stated to begin in adult life, was markedly 
hereditary, and was incurable. I do not see, as has been stated, 
that Waters had noticed the association with dementia. 

The next description, also from the State of New York, is found 
in the American Medical Times for December 19th, 1863, in an 
article on Chronic Hereditary Chorea, by Dr. Irving W. Lyon, 
House Physician, Bellevue Hospital. No mention is made of the 
section of the State in which the cases were found. He states that 
the disease was almost exclusively confined to certain families, 
which were popularly designated the "megrim families." The 
hereditary transmission was fully recognized in the community, 
" and the people among whom it occurs believe this to constitute 
its only legitimate method of propagation, and acting accordingly, 
have repeatedly been known to interdict marriage alliances between 
their children and those believed to be tainted with the megrim 
diathesis under the severe penalty of social ostracism." Dr. Lyon 



CHRONIC PROGRESSIVE CHOREA. 97 

gives instances in which cases occurred through five generations, 
but gives no details, and there was no recognition of the mental 
deterioration. 

In 1872, Dr. George Huntington, of Pomeroy, Ohio (now of La 
Grangeville, N.Y.), published in the Medical and Surgical Reporter 
of Philadelphia, April 13th, an every-day sort of paper on chorea 
minor, at the conclusion of which he described a form which he 
called hereditary chorea, met with at the eastern end of Long 
Island, New York, and well known to both his father and to his 
grandfather, who had practised in that locality. As I have never seen 
Huntington's description reprinted in full, I give it here, calling 
attention merely to the really graphic account he has given in a few 
paragraphs of the salient features of the disease. 

" There are three marked peculiarities in this disease : — 1. Its 
hereditary nature. 2. A tendency to insanity and suicide. 3. Its 
manifesting itself as a grave disease only in adult life." 

1. " Of its hereditary nature. When either or both the parents 
have shown manifestations of the disease, and more especially when 
these manifestations have been of a serious nature, one or more of 
the offspring almost invariably suffer from the disease, if they live to 
adult age. But if by any chance these children go through life 
without it, the thread is broken, and the grandchildren and great- 
grandchildren of the original shakers may rest assured that they are 
free from the disease. This you will perceive differs from the general 
laws of so-called hereditary diseases, as for instance in phthisis, or 
syphilis, when one generation may enjoy entire immunity from their 
dread ravages, and yet in another you find them cropping out in all 
their hideousness. Unstable and whimsical as the disease may be 
in other respects, in this it is firm ; it never skips a generation to 
manifest itself in another ; once having yielded its claims, it never 
regains them. In all the families, or nearly all in which the choreic 
taint exists, the nervous temperament greatly preponderates, and in 
my grandfather's and father's experience, which conjointly cover a 
period of seventy-eight years, nervous excitement in a marked 
degree almost invariably attends upon every disease these people 
may suffer from, although they may not when in health be over- 
nervous." 

2. " The tendency to insanity, and sometimes that form of insanity 
which leads to suicide, is marked. I know of several instances of 
suicide of people suffering from this form of chorea, or who belong 
to families in which the disease existed. As the disease progresses 

H 



98 CHOREA. 

the mind becomes more or less impaired, in many amounting to 
insanity, while in others mind and body both gradually fail until 
death relieves them of their sufferings. At present I know of two 
married men, whose wives are living, and who are constantly making 
love to some young lady, not seeming to be aware that there is any 
impropriety in it. They are suffering from chorea to such an extent 
that they can hardly walk, and would be thought, by a stranger, to 
be intoxicated. They are men of about fifty years of age, but never 
let an opportunity to flirt with a girl go past unimproved. The 
effect is ridiculous in the extreme." 

3. " Its third peculiarity is its coming on as a grave disease, only 
in adult life. I do not know of a single case that has shown any 
marked signs of chorea before the age of thirty or forty years, while 
those who pass the fortieth year without symptoms of the disease, 
are seldom attacked. It begins as an ordinary chorea might begin, 
by the irregular and spasmodic action of certain muscles, as of the 
face, arms, etc. These movements gradually increase, when muscles 
hitherto unaffected take on the spasmodic action, until every muscle 
in the body becomes affected (excepting the involuntary ones), and 
the poor patient presents a spectacle which is anything but pleasing 
to witness. I have never known a recovery, or even an amelioration 
of symptoms in this form of chorea ; when once it begins it clings 
to the bitter end. No treatment seems to be of any avail, and indeed 
now-a-days its end is so well known to the sufferer and his friends, 
that medical advice is seldom sought. It seems at least to be one 
of the incurable." 

Subsequent to Huntington's description but very little is found in 
the literature until 1884, when Ewald 1 called attention anew to the 
question, and reported two cases. In this country Clarence King 2 
(of Machias, N.Y.) reported another family. Then followed in 
rapid succession in a few years many observations by Peretti, 3 
Huber, 4 Hoffmann, 5 Lannois, 6 and others. In 1889 appeared the 
monograph by Huet, 7 in which the historical details and the histories 
in full of the reported cases to that date are given. Since this 
publication there have been numerous additional articles, of which 
the following are the most important : — 

1 Zeitschrift f. Klin. Med., Bd. 7, supplemental Heft, 1884. 

2 New York Medical Journal, 1885, Vol. XLI. 

3 Berliner Klin. Wochenschr., 1885, p. 824. 4 Virchow's Archiv, Bd. 108. 
5 Virchow's Archiv, Bd. III. 6 Revue de Medicine, 1888. 

7 De la C/iorJe Chronique. Paris, 1889. 



SYMPTOMATOLOGY. 99 

Suckling, 1 Diller, 2 Bower, 3 Biernacke, 4 Hay, 5 Jolly, Remak, 7 
Cirincione and Mirto, 8 Mendel, 9 Sinkler, 10 Reynolds, 11 Greppin, 12 
Dreves, 13 Schlesinger, 14 Phelps, 15 Lannois and Chapnis, 16 Ruffmi, 17 
Kronthal and Kalischer, 18 Oppenheim and Hoppe, 19 and Menzies. 20 

Symptomatology.— I will in the first place give the history of 
the cases which have been under my personal observation in two 
families, 21 which illustrate well the hereditary nature of the malady. 

Family X. — First Generation. 

A. B., an Englishman, married C. D., a native of County, 

State of , and had issue eleven children. A. B. died aged 87, 

and his wife aged 85. Neither of them, so far as is known, dis- 
played any mental or bodily peculiarities. Two of the eleven 
children died choreic and demented. 

Of the other children, two of the girls married N.'s. One died 
aged 75, leaving children, all of whom are in good health ; the 
other, Mrs. N., still lives, aged 77, and has healthy children ; 
George died aged 70, a bachelor ; Sarah died aged 50, of typhoid 
fever, without issue ; William died aged 76, leaving a large family, 
none of whom have shown any symptoms of the disease ; Mary 
died of an acute illness, aged 55, leaving healthy issue ; Jane died 
aged 70, leaving a family, none of whom are affected ; two other 
daughters died maidens, well advanced in life. The two affected 
children were James and Margaret. 

James, the first to become affected, began to exhibit remarkable 
muscular irregularities before he was 40. Dr. Ellis writes : " I 
very well remember, in my earliest youth, his grotesque movements, 
exciting unusual attention, and I fear more ridicule than sympathy. 

1 British Medical Journal, 1889, II. 

2 American Journal of the Medical Sciences, 1889, II. 

3 Journal of Nervous and Mental Diseases, 1 890. 

4 Berliner Klin. Wochenschrift, 1S90. 6 University Medical Magazine, 1S90. 
6 Neurologisches Centralblatt, 189 1. 7 Ibid. 8 Ibid. 9 Ibid. 

10 Medical Record. New York, March 12th, 1892. u Medical Chronicle, 1892. 
12 Neurologisches Centralblatt, 1892. 13 Ibid. 

14 Zeitschrift fiir Klin. Medicin, Bd. XX. 

15 Journal of Nervous and Mental Diseases, 1892. 

16 Quoted in Neurologisches Centralblatt, 1893. 

17 Quoted in Revtie Netirologique, 1893. 13 Neurologisches Centralblatt, 1S93. 

19 Archiv fiir Psychiatrie, Bd. XXV. 

20 Journal of Mental Science, Vols. XXXVIII. and XXXIX. 

21 These have already been published in a paper " On Chronic Chorea." Journal 
of Nervous and Mental Diseases, 1893. 

H 2 



IOO CHOREA. 

His swaying, jerking, and fantastically irregular walk compelled him 
from the sidewalk to the unobstructed roadway. Notwithstanding 
his infirmity, he was a great pedestrian, frequently walking from his 
home, eight miles distant, and returning the same day. His sudden 
stops and precipitate advance, his facial contortions and mobile 
features, I recall with great vividness after forty years." His wife 
died in childbed. 

Margaret married J. M. Her symptoms began to develop before 
she was 40. She continued to go about until a few days before her 
death, which occurred in her 65th year. Except a short time before 
her death, she was not entirely helpless, nor were the mental 
symptoms very strongly marked in her case. 

Second Generation. 

Margaret M., the last-mentioned patient, had five children, two of 
whom have already died of the disease, and three are in various 
stages of it. I have seen two members of the family, and have per- 
formed a post-mortem on a third : — 

First child, male, now in his 61st year. A year ago the first 
evidences began. "A man of some character, it is but charity to 
ascribe the eccentricities of his life to disturbed mentality. He 
married twice, but had issue only by his first wife. Several children 
died in infancy, but one surviving is now in good health." This 
patient I could not see. 

Second child, female, married, became choreic in her 40th year, 
and died demented in December, 1890, in her 58th year. She was 
confined to her bed for nearly a year before her death, which 
occurred in the Pennsylvania Hospital for the Insane, Norristown. 
She had four children — three girls and one boy ; all are living and 
in good health, the oldest being now in her 32 nd year. 

Third child, male, aged 55. I saw this patient with Dr. Ellis. 
He has enjoyed good health, and has been able to attend to his 
business until recently. When about 42 he began to get nervous. 
Irregular locomotion was the first symptom ; his speech became 
affected about a year ago. He will make use of a nod or a grunt in 
place of words whenever he can. Lately he has been confined to 
the house, and has been obliged to abandon business. He is very 
irritable, and is steadily passing into a state of dementia. He has 
had five children : four are living and in good health, the oldest 
about 33 years of age ; one died of basilar meningitis at 16. I saw 
this patient in April, 1889, and made the following note : — 



SYMPTOMATOLOGY. 1 01 

Bony, well-built man ; face has an intelligent expression. The 
gait is very peculiar ; he sways from side to side ; the movements 
are irregular, very unlike those of an ataxic, but resemble rather 
those of an alcoholic. He does not use a cane ; in walking the 
feet are not specially spread ; the eyes are not directed to the 
ground. He can stand with his heels together, and with his eyes 
shut ; there are no movements of the hands or arms when at rest, 
but in attempting to move there are large irregular sweeps of the 
arms, and slight tremor. He has great difficulty in feeding himself, 
and sometimes takes two hours or more at a meal. He still can 
write, though with increasing difficulty. He can sign his name, but 
the pen, in forming the letters, is often jerked up and the signature 
is very irregular. With the eyes shut he touches the nose or ear 
with precision and quickly. The grasp of the hand is firm and 
strong. There is no disturbance of sensation, no numbness or 
tingling. The knee-jerks are slightly increased ; the ankle clonus is 
not obtainable. The pupils are of medium size, and react to light 
and on accommodation. The speech is slow, and interrupted fre- 
quently by the interjection ' Hem, ha ! ' This peculiarity, his wife 
says, is of comparatively recent development. The mental condi- 
tion is apparently good ; perception clear. When questioned, how- 
ever, on several occasions, it seemed to take him some time to 
understand our wishes. He takes an interest in what is going on ; 
reads a good deal, particularly the newspapers. 

Within the years which have elapsed since making this note he 
has steadily declined mentally and bodily. 

Fourth child, female, aged 43, married, has had five children. 
One died of scarlet fever ; the others are living, the oldest a boy of 
23. In this case the disease has progressed with greater rapidity 
than in the others, and certain indications of it have been present, 
according to the doctor, since her thirty-fourth year. The mental 
symptoms were first to appear. In April, 1889, I made the follow- 
ing note : — 

Slightly built, somewhat anaemic woman ; she talks clearly and 
rapidly, but occasionally displays a certain childishness, and the 
doctor, who has not seen her for some years, was much struck with 
the change in this respect. 

While sitting quietly there were no irregular movements of her 
limbs, but occasionally there was a slight jerk of the finger, the 
shoulders would move, and once or twice, while speaking, there 
appeared to be irregular contraction of the facial muscles. There 



102 CHOREA. 

is no tremor of the tongue, and the pharyngeal muscles act normally; 
the grasp is good ; she can use her fingers for delicate movements, 
and can thread a needle, and there does not appear to be the slightest 
inco-ordination. The most marked change appeared to be noticed 
in her gait. She walks with the feet somewhat spread, and sways, 
though she follows a straight line fairly well ; she turns with a little 
difficulty, and, if rapidly, loses her balance. Her head is carried 
somewhat stiffly in walking ; she does not trip, and she walks in the 
dark quite well. She stands with the eyes shut and the feet together 
without swaying. 

The muscular power of the legs is good; the knee-jerks are increased 
on both sides ; there are no disturbances of sensation ; the special 
senses are normal ; the pupils are of medium size and react to light 
and on accommodation. In the years since the preceding note 
was made she has lost ground rapidly, and the muscular inco-ordina- 
tion has become much worse. She is now confined to the house, 
and for the greater part of the time to her bed. 

Fifth child, female, aged, at the time of her death, 5 1 ; married ; 
had eight children. Dr. Ellis writes : " After the birth of the 
seventh child, in her thirty-second year, her husband noticed the 
beginning of the trouble in jerking movements of the legs when 
sitting, and when erect she had a trick of raising her heels suddenly 
and standing upon the balls of the toes. Irregular movements of 
the arms speedily followed. When I saw her first, in 1880, she 
could walk a mile or two without apparent fatigue, and would insist 
on walking to church, nearly a mile distant, repelling the suggestion 
that she could not walk as well as another. At this time, in walking, 
her body would be bent forward, her head jerking, with a pendulum- 
like motion, to and fro, and her legs making such irregular and large 
movements that she would make wide excursions on the sidewalk. 
A year later she could no longer go out without assistance. Her 
speech indicated marked changes very early, in her fortieth year, 
and this was (in 1881 and 1882) accompanied by great difficulty in 
swallowing and frequently with alarming spells of strangling. She 
was a most pitiable sight. She suffered also from procidentia uteri ; 
yet in June, 1883, in her forty-third year, she was delivered of her 
eighth child, which survived but a few days. Her menses were per- 
fectly regular, the menopause not occurring until the forty-eighth 
year. Six months before her death she was confined to her bed, 
utterly helpless, and was fed with a spoon. She was now entirely 
demented. 



SYMPTOMATOLOGY. IO3 

" The deep reflexes were rather exaggerated. She could go about 
the house at night with as little help as in the daylight. She was ex- 
ceedingly irritable and cross. The choreic movements stopped in 
sleep ; there was no palsy of the sphincters. Of her eight children, 
seven are living, the oldest in her thirty-third year ; all are in good 
health." 

Postmortem (about thirty hours after death). — Considerable 
wasting of the body ; no enlargements of joints ; no abnormal 
position of limbs ; face a great deal wasted, presenting several 
recent scars and abrasions, the result of falls. 

• The skull-cap was of moderate thickness ; the dura was tense ; 
the meningeal vessels looked stiff ; the longitudinal sinus contained 
recent clots. On the exposed cortex cerebri the arachnoid was some- 
what turbid, and universally separated from the pia by a considerable 
amount of serous exudate; this was especially marked over the 
sulci. The Pacchionian granulations were numerous ; the cortical 
veins moderately full. At the base the arachnoid was turbid, and 
the larger arteries a little stiff; the meninges were not specially 
adherent, and the pia could be stripped without tearing the sub- 
stance. Superficial examination revealed no areas of softening, and 
no special lesions of hemispheres or of cerebellum. There was 
general wasting of the convolutions, which were also, on section, 
rather firm. The gray matter was dark, and in places looked thinner 
than normal. The crura presented no signs of descending degenera- 
tion ; the pons and medulla were natural-looking ; anterior pyramids 
had a clear, normal aspect ; the ventricles were not distended. The 
spinal cord was firm ; arachnoid a little opaque ; pia normal. 
Transverse sections showed no systemic degenerations ; the gray 
matter had a rosy red tint. 

Microscopical Examination. — I am indebted to Dr. Gray for an 
extensive series of sections from various parts of the brain and cord. 
The changes may thus be summarized : The arteries were thickened 
and in places showed hyaline degeneration ; in the smaller arterioles 
fatty changes were very marked in the fresh specimens from the 
cortex. Here and there the perivascular lymph-spaces were large 
and contained leucocytes. The ganglion cells in many sections 
showed very slight changes, not more than are often seen in chronic 
disorders associated with atrophy of the convolutions. There was 
the common vacuolation, and many cells seemed laden with 
pigment. The increase in the connective-tissue elements was more 
evident to the touch and on section than microscopically. Sections 



104 CHOREA. 

of the pons and medulla showed no special foci of disease. Beyond 
thickening of the arteries and a shrinkage in the cells of the 
anterior cornua (probably an artificial change), the sections of the 
cord showed no lesions. 

Neiter Family. 

So far as can be ascertained only four members of the family 
have been affected, namely, mother and three children, one of 
whom was our patient, Peter. 

i. The mother, a German, is stated to have had trouble of the 
same kind as that which Peter has. For many years she made wild 
inco-ordinate movements with her arms, and toward the end of her 
life she could not eat alone and had to be fed. Her mind, also, 
became very weak. The exact duration of the disease in her case 
could not be obtained, but it extended over many years. She is 
said to have died of heart disease. She has one brother living, 
aged 83, who is said to have the disease, but Dr. Chas. Simon, 
who visited him, reports that he is the subject of ordinary senile 
tremor. No information is available with reference to her family. 
Her maiden name was Schmidt. She had four children, of whom 
three have been affected with the disease. 

2. Lizzie N. was well to her 37 th year. Married and had six 
children, of whom two died and four are living and well. After the 
birth of the last child, the chorea developed, beginning first in the 
arms. Her husband noticed that she frequently dropped things. 
The trouble gradually became worse. Her mind became seriously 
affected, she talked incoherently, and had strange ideas. She once 
tried to commit suicide by jumping out of a window. During the 
last year of her life she was helpless, and could not walk alone. 
She died in her 49th year, about twelve years after the first onset of 
the symptoms. Her husband, from whom these facts were obtained, 
says that the disease was called St. Vitus' dance. 

3. Nicholas Neiter, aged about 40, blacksmith, living at Edge- 
wood, Hartford Co., Md. He was seen for me by Dr. Chas. 
Simon, who reports that he is evidently subject to the disease, 
as he displays grotesque inco-ordinate movements of the legs, 
arms, and face. Mentally, too, he is inclined to be childish 
and is very emotional. He regards himself, however, as in a 
condition of perfect health and not affected in any way as his 
brother Peter. 

4. Peter Neiter, aged 59, German, a butcher, was admitted to 



SYMPTOMATOLOGY. 105 

Johns Hopkins Hospital, 1 October 9, 1890. Patient has been in 
this country since 1850. He has always enjoyed good health with 
the exception of malaria when he first came to this country ; he has 
not had syphilis. He dates the present trouble from an attack of 
gastro-intestinal disturbance eight years ago, which followed the 
drinking of large quantities of iced lemonade. At this time he had 
also pains in the head, and he speaks of the occurrence of some- 
thing bursting in his body like a cannon. The movements began 
about five days after this over-heating and taking iced drinks. They 
did not start at any particular part of the body, but were general 
from the outset. They have gradually become worse, particularly 
when voluntary movements are made. They are severe enough to 
prevent him from working, and he has not been able to do much 
for six or eight years. He has fallen sometimes, owing to the 
irregular movements of the legs. He has never at any time lost 
consciousness. Emotion or fright always exaggerates the move- 
ments. He has not had headaches, and, as a rule, sleeps well. His 
appetite has been good and general health excellent. Ever since 
the attack, eight years ago, he has been liable to a recurrence of the 
vomiting whenever he takes cold drinks. He says his memory is 
quite good. He does not think that his speech has been affected. 

Present Condition. — The patient is a large, well-nourished, well- 
built man. The face in repose looks intelligent, but on smiling, 
the expression is fatuous. He answers all questions readily and 
freely ; gives a good account of his condition, and it is more in his 
expression and general behaviour that an indication is found of 
mental impairment. 

When sitting in a chair, at ease, the arms and hands are in more 
or less constant irregular motion. The fingers are extended and 
flexed alternately ; sometimes only one, sometimes the entire set. 
At other times the whole hand will be lifted, or there are constant 
movements of pronation and supination. For half a minute or 
so they may be perfectly motionless. The head and trunk present 
occasional slow movements ; in the latter more of a swaying 
character. The legs jerk irregularly and the feet are flexed or 
extended ; but the movements are not so frequent as in the arms. 
The face in repose is usually motionless, but the lips are occasionally 
brought together more tightly and the chin elevated or depressed. 
There is an occasional movement of the zygomatic and of the 

1 The patient was shown at the Hospital Medical Society, and is reported in 
the Hospital Bulletin, Vol. I. 



106 CHOREA. 

frontal muscles. He puts out the tongue, with tolerably active 
associated movements of the face, and it is usually quickly with- 
drawn or rolled from side to side. It is impossible for him to hold 
it out for any length of time. There are no irregular movements of 
the palate muscles. 

He walks with a curious irregular gait, displaying distinct inco- 
ordination, swaying as he goes, hesitating a moment in a step, keep- 
ing the arms out from the body and in constant motion. The legs 
are spread wide apart ; the steps are unequal in length and he 
seems rather to drag the feet. He stands well with the heels close 
together and the eyes shut. 

There is a suggestion of stiffness about the gait and about the 
way in which he uses his legs. 

The sensation is unaffected. The deep reflexes are increased. 
There is slight ankle clonus, the knee-jerks are exaggerated, and there 
is slight increase in the arm-reflexes. 

The special senses are unimpaired. The pupils are of medium 
size — the right a little larger than the left ; they react to light and 
on accommodation ; there is no nystagmus. He has no fever ■ the 
bowels are regular, and the urine shows no special changes. 

Special Features. — The onset is gradual in the hereditary form ; 
but there are instances, as Observation XL in Huet's monograph 
and Berkeley's case, 1 which followed emotional causes and came on 
suddenly. 

The earliest manifestations are motor. It is first noticed that the 
gait is not quite so steady as usual, or that there are slight irregular 
movements of the hands. In a few cases the mental disturbance 
has been the first symptom, but, as a rule, there is no perceptible 
disturbance in the intellectual faculties until the motor features of 
the disease are well developed. In the three cases which I saw in 
the Family X the disturbance in locomotion was the first symptom 
observed. 

Character of the Movements. — Though irregular, involuntary and 
arrythmic, the movements in the chronic progressive form differ 
in one important particular from those of Sydenham's chorea, 
namely, in the absence of the brusque, quick, jerking character. 
At first indeed the condition is one rather of muscular instability 
or inquietude, and when the patient is at rest there are irregular 
movements of the muscles of the hands or arms, which perhaps 
scarcely alter the position of the limb, or slight, slow contractions 
1 Medical News, 1883, Vol. XLIII. 



SPECIAL FEATURES. 107 

pass over the muscles of the face. There may be no movements of 
the hands or arms when in repose, but any attempt at grasping an 
object may be associated with large, irregular, sweeping movements, 
as were present in a very striking manner in the third child in the 
second generation of Family X. In this case and in his sister the 
movements of the arms were rather those of inco-ordination than 
truly choreiform, and yet such a voluntary effort as the threading of 
a needle could be performed by the latter. In fact the two points 
of difference in the movements from those of Sydenham's chorea 
are the slowness and the inco-ordination. The slowness of move- 
ment is noticed particularly in the facial grimaces, which I have 
never seen presenting that rapidity so characteristic of chorea 
minor. These differences have not always been appreciated by 
writers. Thus E. S. Reynolds and Menzies state that the move- 
ments are exactly like those of ordinary chorea. Doubtless, the 
cases differ somewhat in this respect, but in those which have come 
under my observation the points of contrast above referred to were 
very manifest. 

One of the most striking peculiarities is the gait, which, as in the 
members of Family X, may be very early affected. It has been 
best described by King, who states very correctly that when fully 
developed it constitutes one of the most important features of the 
disease, and is unlike the gait in any other affection. The station 
may be good with the exception of a slight swaying of the trunk, 
but on attempting to walk the unsteadiness develops and is cha- 
racterized by large lateral deviations from the straight line, by 
marked swaying of the body and sometimes by precipitate move- 
ments, in which the patient almost falls but catches himself. As 
mentioned by Dr. Ellis, even when this gait is well developed the 
patient may be able to take long walks. It has been very well com- 
pared with the gait of a drunken man. The difficulty in locomo- 
tion may persist for many years before the patient becomes bed- 
ridden. 

In a majority of the cases speech is affected, at first manifested in 
a slowness and hesitancy, and in the interjection of such expressions 
as hem ! ha ! &c, and finally by great indistinctness, owing to 
inability to pronounce the words clearly. It has not the staccato, 
scanning quality of the speech of multiple sclerosis. 

The handwriting is early affected ; the letters are badly formed, 
irregular, and run into each other, and the lines are not straight, but 
with large zig-zags. In the later stages writing becomes impossible. 



108 CHOREA. 

Under the influence of emotion, as during examination, the 
movements are usually aggravated. The influence of the will may 
control them to some extent in the earlier periods of the disease, 
but, as a rule, when the disease is well developed, the movements 
are not influenced by any voluntary effort of the patient. One 
member of the Family X, in whom the disease was quite evident, 
could thread her needle without difficulty. 

Sensation is not affected, nor are the special senses involved. 
The muscular force is retained until the disease is well advanced. 
The reflexes are usually increased, and the knee-jerks may be 
exaggerated at an early stage, as in the fourth child in Family X. 

Huntington gave as one of the three special features of the 
disease " a tendency to insanity and suicide." This has been con- 
firmed by subsequent observers. At first there may be only 
irritability or moodiness and depression, but the most constant 
change is a slow but progressive enfeeblement of the mental 
faculties, without, as a rule, hallucinations, or delusions of grandeur 
or of persecution. The disposition to suicide has also been observed 
by many writers since Huntington. 

Heredity is one of the most remarkable features of the disease ; 
indeed, in the whole range of inherited disorders there is scarcely 
one in which a larger percentage of individuals have been found 
affected. There are families in which 25 per cent, of all the 
members known have been attacked, and more than 50 per cent, of 
adults. The tendency appears to be transmitted through male and 
female alike, and the sexes are about equally attacked, though in 
some families the males have suffered most severely. Isolated cases 
occur in members of perfectly healthy families, so that the designa- 
tion chronic progressive chorea, which has been recommended, is 
more correct than that usually employed, and which lays the chief 
stress upon heredity. The age of onset, the third feature which 
Huntington regarded as characteristic, is usually, but not always, in 
middle life. In a very large proportion of the cases with an 
hereditary taint, the disease does not begin until after the 30th year. 
Huet gives seven instances of this form, in which the disease began 
before this date. In Hoffmann's patient the affection began with 
adolescence. 

Morbid Anatomy and Pathology.— Only a few cases of the 
hereditary form have been carefully studied. In Huber's case, in 
which the disease had lasted for eight years, there was pachy- 
meningitis and chronic adhesions between the pia and the cortex. 



MORBID ANATOMY AND PATHOLOGY. IOp 

In the sisters described by MacLeod, 1 in one case which had lasted 
about six years there was pachymeningitis with hsematoma on the left 
side, and atrophy of the convolutions ; the pia mater was adherent, 
and the ventricles were somewhat dilated. In the other case the 
chorea had come on late in life, about the 70th year, and death had 
followed in the 73rd year. The autopsy showed fibrous tumours of 
the dura mater on the left side, chiefly in the neighbourhood of the 
bases of the first and second frontal convolutions. In a case 
examined by Klebs, 2 in addition to haemorrhagic pachymeningitis, 
he found foci of cell infiltration in the white matter, and hyaline 
thrombi in the vessels. 

Greppin 3 has contributed a very careful study of a case from 
Professor Wille's clinic at Basel. There was a moderate grade of 
atrophy of the convolutions, and microscopically a condition of 
chronic encephalitis, consisting chiefly of small and large foci of cell 
infiltration, particularly in the superficial white matter in the frontal 
and temporal lobes. 

Kronthal and Kalisher's 4 study of a case which had lasted for 
fifteen years is particularly thorough. They found chronic pia- 
arachnitis with adhesions of the membranes to the cerebral cortex, 
particularly in the frontal region and over the central gyri ; thicken- 
ing of the vessel walls and small celled infiltration about them ; 
diffuse degeneration of the pyramidal tracts of the pons, medulla, and 
antero-lateral columns of the cord, with other changes of less moment. 
The authors were not prepared to assert that any of these conditions 
were connected causally with the chronic progressive chorea. 

In the monograph of Huet, eight autopsies are given in cases of 
chronic chorea without hereditary basis. In the case reported by 
Macleod, the man had had chorea for six years with dementia. 
There were pachymeningitis and hematoma of the dura mater on 
both sides. In Maclaren's case, the man, aged 38, had had chorea 
for eight years and progressive dementia. There was thickening of 
the membranes with atrophy of the convolutions. In Berkeley's 
case, a man, aged 41, had had chorea for seven years and gradual 
dementia. There was atrophy of the dura, and microscopically 
dilatation of the arteries with thickening of the walls, small areas of 
softening, and vacuolation of the nerve cells. In the case reported 
by Bacon, the woman, aged 61 had had chorea for three years. 

1 Journal of Mental Science, 18S1. 

2 Cori'espondenzblatt f. Schweitzer Aertze, iSSS. 

3 Loc. cit. 4 Loc. cit. 



IIO CHOREA. 

There was atrophy of the cortex with a chronic arachnitis. In the 
case of Vassitch, a woman, aged 41, had had chorea for seven years 
with gradual mental impairment, and delusions of persecution. She 
had previously had hysteria. No lesions were found at the autopsy. 
In the case of Pau, a man aged 55 was admitted to the asylum, 
and died in six months. There was atrophy of the cortex with 
increased density. In Tissier's case, a woman, aged 79, had had 
chorea for two years with mental enfeeblement. The pia mater was 
a little thickened, was only adherent where there were some spots of 
cortical softening, which were chiefly in the external portions of the 
occipital lobes, and the second and third frontal convolutions of the 
left hemisphere. 

The most recent contribution is by Oppenheim and Hoppe, 1 who 
have studied two cases ; the first a woman, aged 55, in whom the 
disease had lasted from her thirty-second year. There was hydro- 
cephalus externus, and turbidity of the membranes ; the convolutions 
were atrophied and the sulci deep. The ventricles were not enlarged. 
A disseminated encephalitis was manifest by the presence of foci of 
small round cells, chiefly in the central convolutions, and there was 
atrophy of the layer of small round cells in the grey cortex. There 
was some diffuse sclerosis also in the antero-lateral columns of the 
cord. In the second case, which died at the age of 75, the disease 
began late in the seventieth year. A chronic pachymeningitis 
hemorrhagica was found with moderate atrophy of the convolutions, 
and in the region of the central convolutions there were foci of 
miliary infiltration and atrophy also of the small round cells in the 
border between the first and second cortical layers. From their 
investigation, with which those of Golgi, Klebs, and Greppin 
practically agree, they regard the disease anatomically as a cortical 
and sub-cortical miliary disseminated encephalitis. 

Menzies 2 describes one autopsy in which he found atrophy of the 
cortex cerebri, with increased consistence, and microscopically coarse 
neuroglia, thickened vessels and degeneration of the nerve cells of 
all the layers. In brief the changes belong to those degenerative 
processes in the grey cortex which are common to so many affections 
associated with impaired motor and psychical functions. 

So far nothing has been found which is peculiar to the disease or 

in any way specific. Of the manner in which these changes are 

initiated, of the nature of the first slight departure from the normal, 

of the character of the differences in the morbid processes which 

1 Loc. cit. 2 Lor. cit. 



MORBID ANATOMY AND PATHOLOGY. Ill 

make the external picture that of chronic progressive chorea and not 
that of, say, double athetosis, why in the members of certain families, 
after a certain age, the malady is liable to occur, why other indi- 
viduals cast in the same family mould should escape — of these and 
other questions science at present offers no solution. 

Diagnosis. — The recognition of chronic progressive chorea, so 
easy in the family form, becomes exceedingly difficult in cases with- 
out this peculiarity. There can be no question, in the first place, 
that cases with and cases without hereditary features present identical 
symptoms and course. The term may be used to group the cases 
which have a chronic and progressive course. The heredity, as in 
Freedrich's ataxia, is only one, and an inconstant feature of the 
disorder. The " progress " is towards motor and mental enfeeble- 
ment. 

The cases of chronic chorea dating from birth, or shortly after, 
almost invariably have spasm as an associated feature, a symptom 
which excludes a case, I believe, from the category under considera- 
tion. In the later stages, after many years, there may be increased 
reflexes, and some rigidity in chronic progressive chorea, but it does 
not form a feature in the fully-developed disease. 

The following are good illustrative cases of the so-called con- 
genital and spastic chorea 1 : — 

Case 1 6. — Mary M , aet. 4. Breech presentation, delay at the 

head ; was six hours before she was resuscitated. Began to talk at 
two years ; never walked ; almost from birth she has had peculiar 
movements of hands and arms ; the thumbs are turned in and 
there is constant irregular motion of the arms and hands, with 
stiffness, which is made worse when she attempts to control it ; it is 
like a chorea. There is also some co-ordination of the head. She is 
well nourished ; no wasting. Co-ordination of legs good ; but she 
does not walk. 

Case 17. — Nellie P , aet. 9. Parents healthy, five children 

dead of seven. Seven years ago had fits while teething, had fits 
constantly for twenty-one days ; for nine months had seven to nine 
per diem ; in very weak health when fits ceased. Presejit state : 
Speech hesitating; memory not affected; unable to stand, sit, 
feed herself, or assist herself in any way ; can move every muscle 
in the body, but with an irregular movement which prevents 
her using any group of muscles ; the movement is choreoid ; in 
attempting to grasp an object the fingers are thrown out in a stiff, 
1 From my " Cerebral Palsies of Children," p. 66. 



112 CHOREA. 

spasmodic, and irregular manner, and she is unable to close them 
over the object. 

So also from double athetosis, certain forms of which resemble 
closely chronic progressive chorea, the element of spasm is the most 
important differential sign. As Audry, in his admirable monograph 
on Double Athetosis, 1 says: — The athetoid movements in the limbs 
are associated with rigidity, even with contracture of the muscles. 
It is a symptom essentially spasmodic, the reflexes are found 
exaggerated when the spasm of the muscles is sufficiently easy to 
permit of the trial. The gait also is spastic. 

From the various forms of chronic generalized tic there could 
rarely be any difficulty, the character of the movements are so 
radically different. Charcot, however, gives a case 2 of tic which 
simulated somewhat Huntington's chorea. 

With the exception of Charcot, and his pupil, Huet, all the 
writers on chronic progressive chorea regard the disease as totally 
different from chorea minor, a view which seems to me just, when 
we take into consideration the clinical features, particularly the 
character of the movements, the progressive course, the heredity, and 
the anatomical lesions. 

Unhappily the treatment of chronic progressive chorea is futile. 
In the words of the great physician, whose loss all clinical physicians 
deplore, " son evolution est fa tale, et la therapeutique est impuis- 
sante a l'arreter, ne fut-ce qu'un instant, dans sa marche progres- 
sive." 

1 U Athitose Double. Paris, 1892. 

2 Legons du Mardi, 1888-89, P- 4^3- 



APPENDIX. 



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ii4 



APPENDIX. 



Qg. d 






G l 



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APPENDIX. 



115 



*e o\ 
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Sf S°9 



5j K On 

^3 ^5 00 

Pi ;s<» 



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c 

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n6 



APPENDIX. 











































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1=1 


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APPENDIX. 117 









»S « ^ "■> 






<^£ 



rxS 



<; u h £ < o K 



a 



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a, 



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c ^i-.y .-.si's ±i B •'i n. -£• a 

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n8 



APPENDIX. 



o 

a 


8 

CO - 
in 


fettiva delta Corea del 
Sydenham, Napoli, 
1893 


If 


CM 

ON 


(0 

0) ~ 






och, Deutsches 
Archiv f. Klin. Med. , 
Bd. xl. 




s 




O 

Q 




O 

Q 




Wollenberg, Archiv f. 
Psych, and Nerven- 
krankheiten, Bd. 
XXIII. 




s 






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cd 
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APPENDIX. 119 



j"p *> "£ hh o o o o s"^§ 



G^ 



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kj t-H 









If- 

^j <u - 

o^>d se o g -5 "^y 3 g -^= .c • "£ c n =? 



ni 


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aj 






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& 03 Ph <| U O 



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2 2 1-3 S S 2r S '- 

.-£ .-s ^S ,0 shij flti .-a 8^ 

s s o a & u o s k_ 

^ « •■* t If} » fH I W 

t«-a is Is r- -S I 



120 



APPENDIX. 





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2 


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CO N il J S 




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APPENDIX. 



121 



*> 


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^ 


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s 


5 








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^ 


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cu 




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i5> m 


o 










cu 
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-*> 


c 
o 

en 

CU 




o 

u 
C3 


1j 

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"3! N 

03" S *» 

33 ^d • 
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3 M W 


f-i 




Ph 




U 




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s-< 










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u 

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o 


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d 
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u 






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INDEX. 



Arithmomania, 87 
Athetosis, double, 112 
Auditory hallucinations in tic, 84 

Balancing, 89 

Barking cough of puberty, 95 

Blind, forms of tic in the, 89 

Chorea, canine, 17, 63, 68 

chronic progressive, 96 

diagnosis of, III 

gait in, 107 

heredity in, 97, 108 

handwriting in, 107 

insanity in, 97, 108 

morbid anatomy of, 108 

pathology of, no 

speech in, 107 

suicide in, 97, 108 

symptoms of, 99, 106 

congenital, 2, 39, III 

corpuscles, 62 

electric, 75 

Huntington's, 96 

insaniens, 22 

hysterical, 2, 18 

major, 2 

Choree malleatoire, 19 

Chorea minor, 3 

acute forms of, 21, 22, 39 

in adults, 44 

in the aged, 44 

age incidence of, 6 

anaemia and, 17 

apoplexy in, 61 

arsenic, use of, in, 70 

arthritis and, 13 

atrophy of muscles in, 32 

bacteriology of, 63 



Chorea minor, blood in, 18, 60 

baths, use of, in, 70, 71 

cutaneous symptoms of, 35 

chlorosis and, 18 

definition of, 2 

delirium in, 28, 35 

diet in, 70 

duration of, 39 

embolic theory of, 61 

endocarditis in, 30, 32, 33, 

60 

epilepsy and, 34 

eye-strain and, 1 1 

face, affection of muscles in, 

29 

expression of, in, 35 

family disposition in, 9 

fatal cases of, 51, 60, 113 

feeding in, 70 

fever in, 37 

fright as a cause of, 10 

geographical distribution of, 9 

hands, affection of, in, 29 

haematuria in, 37 

haemorrhage as a factor in, 

18 

headache in, 20, 34 

herpes zoster in, 35 

hemiplegic weakness in, 31 

heredity in, 10 

heart in, 47 

historical note on, 3 

hysteria and, 18 

infection, theory of, in, 64 

imitation in, 10 

infectious diseases and, 16 

incontinence of urine in, 30 

insanity in, 35 

joints in, see Arthritis. 



124 



INDEX. 



Chorea minor, kidney, affections of, in, 

37 

■ ■ limbs, order of attack in, 29 

locality, influence of, in, 9 

massage in, 70 

mental symptoms of, 34 

micro-organisms in, 63 

■ mild form of, 20 

morbid anatomy of, 60 

muscles in, 32 

muscular weakness in, 31 

naso-pharyngeal irritation in, 

11 

nephritis in, i>7 

neuritis in, 33, 71 

and old age, 44 

optic neuritis in, 34 

■ pains, in, 33 

paralysis in, 31 

pathology of, 64 

pericarditis in, 58 

pigmentation of skin in, 36, 

70 

poisons as a cause of, 19 

post-partum, 44 

■ pregnancy and, 41 

prognosis in, 39 

psychical influences in, 10 

purpura in, 36 

race and, 7 

recurrence of, 40 

refraction errors and, II, 12 

rest, influence of, in, 69 

retinal hyperemia in, 34 

retinal artery, embolism of, 

34 

reflexes, state of, in, 34 

reflex irritation in, 1 1 

rheumatism and, 13, 67 

school life, influence of, 10 

scarlet fever and, 16 

seasonal relations of, 5 

sensation, disturbances of, 33 

severe form of, 21 

sex in, 6 

site of changes in, 68 

sleep, influence of, on, 30 

spasmodic disorders, co-exist- 
ence with, 34 

— speech, disturbances of, in, 29 

sub-cutaneous fibroid nodules 

in, 16 
temperament and, 10 



Chorea minor, temperature, see Fever. 

tic and, 40 

traumatic influences in, 1 1 

treatment of, 68 

urine in, 37 

uterus, irregular contractions 

of, in, 30 

whooping-cough and, 1 7 

mollis, 31 

pseudo-, 2 

rhythmic, 2 

secondary, 2 

spastica, 2, 39, 1 1 1 

Sydenham's, 2 

symptomatic, 2 

Choreiform affections, 2, 72 

Coprolalia, 87 

Cries, involuntary, 86 

Delire du toucher, 88 
" Dervish" tic, 90 
Dubini's disease, 75 

Echolalia, 79, 86 
Echokinesia, 87 
Electric chorea, 75 
Epilepsy and chorea minor, 34 
Epilepsia nutans, 89 
Expiratory cries, 90, 94 

Folie choreique, 35 
Folie pourquoi, 87 

Gait in chronic progressive chorea, 107 

Generalized tic, 75 

Gilles de la Tourette's disease, 79 

Habit spasm, 73 
Habit chorea, 73 
Head-nodding, 89 
Head-banging, 89 
Historical note, chorea minor, 3 

chr. progressive chorea, 96 

Huntington's chorea, 96 
Hysteria and chorea minor, 18 
Hysterical cries, 91, 94 
Hysterical chorea, 2 

Ideas, fixed, 87 
Imperative ideas, 87 
Inspiratory cries, 90, 94 
Involuntary cries, 86 



INDEX. 



125 



Jumpers of Maine, 87 

Krouomania, 89 

Latah, 87 
Limp chorea, 31 
Localized tic, 73 

Maladie de la tic convulsif, 79 
Mania, dancing, 4 

Movements, character of, in chorea 
minor, 29 

in chr. pro. chorea, 106 

— — in tic, 84 

Myriachit, 87 

Obsessions mentales, 87 
Onomatomania, 87 



Pendulum spasm, 90 
Ptussis canina, 95 

Respiratory muscles, spasm of, 90 



Simple tic, 7^ 

St. Vitus' dance, 1, 3, 4 



Tic, definitions of, 72 

forms of, 73 

with coprolalia, &c, 79 

generalized, 75 

localized, 7^ 

simple, 73 

complex co-ordinated forms, 

prognosis of, 88